Prediction of the responsiveness to pharmacological chaperones: lysosomal human alpha-galactosidase, a case of study (493 views)
Orphanet Journal Of Rare Diseases (ISSN: 1750-1172), 2010 Dec 8; 5: 36-36.
Export to BibTeX Export to EndNote
Paper type: Abstract, Conference, Research Support, Non-U. S. Gov'T,
Impact factor: 5.933, 5-year impact factor: 5.968
Url: http://www.scopus.com/inward/record.url?eid=2-s2.0-78649755176&partnerID=40&md5=cca6f679bee731cc9815f901dba39d1e
Keywords: 1-Deoxynojirimycin Metabolism Pharmacology Therapeutic Use, Catalytic Domain, Fabry Disease Drug Therapy, Humans, Lysosomes Enzymology, Models, Molecular, Molecular Chaperones Metabolism Pharmacology Therapeutic Use, Mutation, Predictive Value Of Tests, Software, Structure-Activity Relationship, Treatment Outcome, Alpha-Galactosidase Chemistry Genetics Metabolism, Alpha Galactosidase, 1 Deoxynojirimycin, Article, Enzyme Activity, Enzyme Structure, Gene Mutation, Gene Sequence, Genetic Association, Missense Mutation, Thermodynamics, Thermostability, Chemical Structure, Computer Program, Enzyme Active Site, Structure Activity Relation,
Affiliations:
*** IBB - CNR ***
Istituto di Chimica Biomolecolare-CNR, Pozzuoli, Italy.
High Performance Computing and Networking Institute-CNR, Napoli, Italy
Dipartimento di Biologia Strutturale e Funzionale, Universita' Federico II, Napoli, Italy
Istituto di Biostrutture e Bioimmagini-CNR, Napoli, Italy
References:
Fan, J.Q., Ishii, S., Asano, N., Suzuki, Y., Accelerated transport and maturation of lysosomal alpha-galactosidase A in Fabry lymphoblasts by an enzyme inhibitor (1999) Nat Med, 5, pp. 112-5. , 10.1038/4801. 988384
Khanna, R., Benjamin, E.R., Pellegrino, L., Schilling, A., Rigat, B.A., Soska, R., Nafar, H., Lun, Y., The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of beta-glucosidase Febs J, 277, pp. 1618-38. , 10.1111/j.1742-4658.2010.07588.x. 20148966
Flanagan, J.J., Rossi, B., Tang, K., Wu, X., Mascioli, K., Donaudy, F., Tuzzi, M.R., Porto, C., The pharmacological chaperone 1-deoxynojirimycin increases the activity and lysosomal trafficking of multiple mutant forms of acid alpha-glucosidase (2009) Hum Mutat, 30, pp. 1683-92. , 10.1002/humu.21121. 19862843
Tropak, M.B., Reid, S.P., Guiral, M., Withers, S.G., Mahuran, D., Pharmacological enhancement of beta-hexosaminidase activity in fibroblasts from adult Tay-Sachs and Sandhoff Patients (2004) J Biol Chem, 279, pp. 13478-87. , 10.1074/jbc.M308523200. 14724290
Caciotti, A., Donati, M.A., D'Azzo, A., Salvioli, R., Guerrini, R., Zammarchi, E., Morrone, A., The potential action of galactose as a "chemical chaperone": Increase of beta galactosidase activity in fibroblasts from an adult GM1-gangliosidosis patient (2009) Eur J Paediatr Neurol, 13, pp. 160-4. , 10.1016/j.ejpn.2008.03.004. 18571950
Suzuki, Y., Chemical chaperone therapy for GM1-gangliosidosis (2008) Cell Mol Life Sci, 65, pp. 351-3. , 10.1007/s00018-008-7470-2. 18202827
Parenti, G., Treating lysosomal storage diseases with pharmacological chaperones: From concept to clinics (2009) EMBO Mol Med, 1, pp. 268-79. , 10.1002/emmm.200900036. 20049730
Beck, M., Therapy for lysosomal storage disorders IUBMB Life, 62, pp. 33-40. , 20014233
Loo, T.W., Clarke, D.M., Chemical and pharmacological chaperones as new therapeutic agents (2007) Expert Rev Mol Med, 9, pp. 1-18. , 10.1017/S1462399407000361. 17597553
Fan, J.Q., A counterintuitive approach to treat enzyme deficiencies: Use of enzyme inhibitors for restoring mutant enzyme activity (2008) Biol Chem, 389, pp. 1-11. , 10.1515/BC.2008.009. 18095864
Schaefer, E., Mehta, A., Gal, A., Genotype and phenotype in Fabry disease: Analysis of the Fabry Outcome Survey (2005) Acta Paediatr Suppl, 94, pp. 87-92. , discussion 79. 10.1080/08035320510031045. 15895718
Spada, M., Pagliardini, S., Yasuda, M., Tukel, T., Thiagarajan, G., Sakuraba, H., Ponzone, A., Desnick, R.J., High incidence of later-onset fabry disease revealed by newborn screening (2006) Am J Hum Genet, 79, pp. 31-40. , 10.1086/504601. 16773563
Benjamin, E.R., Flanagan, J.J., Schilling, A., Chang, H.H., Agarwal, L., Katz, E., Wu, X., Desnick, R.J., The pharmacological chaperone 1-deoxygalactonojirimycin increases alpha-galactosidase A levels in Fabry patient cell lines (2009) J Inherit Metab Dis, 32, pp. 424-40. , 10.1007/s10545-009-1077-0. 19387866
Shin, S.H., Kluepfel-Stahl, S., Cooney, A.M., Kaneski, C.R., Quirk, J.M., Schiffmann, R., Brady, R.O., Murray, G.J., Prediction of response of mutated alpha-galactosidase A to a pharmacological chaperone (2008) Pharmacogenet Genomics, 18, pp. 773-80. , 10.1097/FPC.0b013e32830500f4. 18698230
Shimotori, M., Maruyama, H., Nakamura, G., Suyama, T., Sakamoto, F., Itoh, M., Miyabayashi, S., Wataya-Kaneda, M., Novel mutations of the GLA gene in Japanese patients with Fabry disease and their functional characterization by active site specific chaperone (2008) Hum Mutat, 29, p. 331. , 10.1002/humu.9520. 18205205
Monserrat, L., Gimeno-Blanes, J.R., Marin, F., Hermida-Prieto, M., Garcia-Honrubia, A., Perez, I., Fernandez, X., Paya, E., Prevalence of fabry disease in a cohort of 508 unrelated patients with hypertrophic cardiomyopathy (2007) J Am Coll Cardiol, 50, pp. 2399-403. , 10.1016/j.jacc.2007.06.062. 18154965
Dundas, J., Ouyang, Z., Tseng, J., Binkowski, A., Turpaz, Y., Liang, J., CASTp: Computed atlas of surface topography of proteins with structural and topographical mapping of functionally annotated residues (2006) Nucleic Acids Res, 34, pp. 23116-8. , 10.1093/nar/gkl282. 16844972
http://sts.bioengr.uic.edu/castp/index.php, CASTPGarman, S.C., Garboczi, D.N., The molecular defect leading to Fabry disease: Structure of human alpha-galactosidase (2004) J Mol Biol, 337, pp. 319-35. , 10.1016/j.jmb.2004.01.035. 15003450
Guce, A.I., Clark, N.E., Salgado, E.N., Ivanen, D.R., Kulminskaya, A.A., Brumer, H., Garman, S.C., Catalytic mechanism of human alpha-galactosidase J Biol Chem, 285, pp. 3625-32. , 10.1074/jbc.M109.060145. 19940122
Lieberman, R.L., D'Aquino, J.A., Ringe, D., Petsko, G.A., Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability (2009) Biochemistry, 48, pp. 4816-27. , 10.1021/bi9002265. 19374450
Ishii, S., Yoshioka, H., Mannen, K., Kulkarni, A.B., Fan, J.Q., Transgenic mouse expressing human mutant alpha-galactosidase A in an endogenous enzyme deficient background: A biochemical animal model for studying active-site specific chaperone therapy for Fabry disease (2004) Biochim Biophys Acta, 1690, pp. 250-7. , 15511632
Petock, J.M., Torshin, I.Y., Weber, I.T., Harrison, R.W., Analysis of protein structures reveals regions of rare backbone conformation at functional sites (2003) Proteins, 53, pp. 872-9. , 10.1002/prot.10484. 14635129
Cubellis, M.V., Caillez, F., Blundell, T.L., Lovell, S.C., Properties of polyproline II, a secondary structure element implicated in protein-protein interactions (2005) Proteins, 58, pp. 880-92. , 10.1002/prot.20327. 15657931
Worth, C.L., Bickerton, G.R., Schreyer, A., Forman, J.R., Cheng, T.M., Lee, S., Gong, S., Blundell, T.L., A structural bioinformatics approach to the analysis of nonsynonymous single nucleotide polymorphisms (nsSNPs) and their relation to disease (2007) J Bioinform Comput Biol, 5, pp. 1297-318. , 10.1142/S0219720007003120. 18172930
Topham, C.M., Srinivasan, N., Blundell, T.L., Prediction of the stability of protein mutants based on structural environment-dependent amino acid substitution and propensity tables (1997) Protein Eng, 10, pp. 7-21. , 10.1093/protein/10.1.7. 9051729
Cheng, J., Randall, A., Baldi, P., Prediction of protein stability changes for single-site mutations using support vector machines (2006) Proteins, 62, pp. 1125-32. , 10.1002/prot.20810. 16372356
Gromiha, M.M., An, J., Kono, H., Oobatake, M., Uedaira, H., Prabakaran, P., Sarai, A., ProTherm, version 2.0: Thermodynamic database for proteins and mutants (2000) Nucleic Acids Res, 28, pp. 283-5. , 10.1093/nar/28.1.283. 10592247
http://www.informedia.cs.cmu.edu/yanrong/MATLABArsenal/MATLABArsenal.htm, MATLAB-ArsenalAltschul, S.F., Madden, T.L., Schaffer, A.A., Zhang, J., Zhang, Z., Miller, W., Lipman, D.J., Gapped BLAST and PSI-BLAST: A new generation of protein database search programs (1997) Nucleic Acids Res, 25, pp. 3389-402. , 10.1093/nar/25.17.3389. 9254694
Ramensky, V., Bork, P., Sunyaev, S., Human non-synonymous SNPs: Server and survey (2002) Nucleic Acids Res, 30, pp. 3894-900. , 10.1093/nar/gkf493. 12202775
Filoni, C., Caciotti, A., Carraresi, L., Cavicchi, C., Parini, R., Antuzzi, D., Zampetti, A., Garman, S.C., Functional studies of new GLA gene mutations leading to conformational Fabry disease Biochim Biophys Acta, 1802, pp. 247-52. , 19941952
Solis, M.A., Pascual, B., Bosca, M., Ramos, V., Carda, C., Monteagudo, C., Torregrosa, I., Miguel, A., New mutation in female patient with renal variant of Fabry disease and HIV J Nephrol, 23, pp. 231-3. , 20155722
Borglum, A.D., Byskov, A., Cubellis, M.V., Kruse, T.A., An EcoRI polymorphism for the PLAUR gene (1991) Nucleic Acids Res, 19, p. 6661. , 10.1093/nar/19.23.6661-a. 1684424
http://www.hgmd.cf.ac.uk/ac/index.php, HGMDMehta, A.B., Anderson-Fabry disease: Developments in diagnosis and treatment (2009) Int J Clin Pharmacol Ther, 47 (SUPPL. 1), pp. 1966-74. , 20040315
Hoffmann, B., Fabry disease: Recent advances in pathology, diagnosis, treatment and monitoring (2009) Orphanet J Rare Dis, 4, p. 21. , 10.1186/1750-1172-4-21. 19818152
Smith, R.E., Lovell, S.C., Burke, D.F., Montalvao, R.W., Blundell, T.L., Andante: Reducing side-chain rotamer search space during comparative modeling using environment-specific substitution probabilities (2007) Bioinformatics, 23, pp. 1099-105. , 10.1093/bioinformatics/btm073. 17341496
Connolly, M.L., Solvent-accessible surfaces of proteins and nucleic acids (1983) Science, 221, pp. 709-13. , 10.1126/science.6879170. 6879170
Fan, J. Q., Ishii, S., Asano, N., Suzuki, Y., Accelerated transport and maturation of lysosomal alpha-galactosidase A in Fabry lymphoblasts by an enzyme inhibitor (1999) Nat Med, 5, pp. 112-5. , 10. 1038/4801. 988384
Flanagan, J. J., Rossi, B., Tang, K., Wu, X., Mascioli, K., Donaudy, F., Tuzzi, M. R., Porto, C., The pharmacological chaperone 1-deoxynojirimycin increases the activity and lysosomal trafficking of multiple mutant forms of acid alpha-glucosidase (2009) Hum Mutat, 30, pp. 1683-92. , 10. 1002/humu. 21121. 19862843
Tropak, M. B., Reid, S. P., Guiral, M., Withers, S. G., Mahuran, D., Pharmacological enhancement of beta-hexosaminidase activity in fibroblasts from adult Tay-Sachs and Sandhoff Patients (2004) J Biol Chem, 279, pp. 13478-87. , 10. 1074/jbc. M308523200. 14724290
Loo, T. W., Clarke, D. M., Chemical and pharmacological chaperones as new therapeutic agents (2007) Expert Rev Mol Med, 9, pp. 1-18. , 10. 1017/S1462399407000361. 17597553
Fan, J. Q., A counterintuitive approach to treat enzyme deficiencies: Use of enzyme inhibitors for restoring mutant enzyme activity (2008) Biol Chem, 389, pp. 1-11. , 10. 1515/BC. 2008. 009. 18095864
Benjamin, E. R., Flanagan, J. J., Schilling, A., Chang, H. H., Agarwal, L., Katz, E., Wu, X., Desnick, R. J., The pharmacological chaperone 1-deoxygalactonojirimycin increases alpha-galactosidase A levels in Fabry patient cell lines (2009) J Inherit Metab Dis, 32, pp. 424-40. , 10. 1007/s10545-009-1077-0. 19387866
Shin, S. H., Kluepfel-Stahl, S., Cooney, A. M., Kaneski, C. R., Quirk, J. M., Schiffmann, R., Brady, R. O., Murray, G. J., Prediction of response of mutated alpha-galactosidase A to a pharmacological chaperone (2008) Pharmacogenet Genomics, 18, pp. 773-80. , 10. 1097/FPC. 0b013e32830500f4. 18698230
http: //sts. bioengr. uic. edu/castp/index. php, CASTPGarman, S. C., Garboczi, D. N., The molecular defect leading to Fabry disease: Structure of human alpha-galactosidase (2004) J Mol Biol, 337, pp. 319-35. , 10. 1016/j. jmb. 2004. 01. 035. 15003450
Guce, A. I., Clark, N. E., Salgado, E. N., Ivanen, D. R., Kulminskaya, A. A., Brumer, H., Garman, S. C., Catalytic mechanism of human alpha-galactosidase J Biol Chem, 285, pp. 3625-32. , 10. 1074/jbc. M109. 060145. 19940122
Lieberman, R. L., D'Aquino, J. A., Ringe, D., Petsko, G. A., Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability (2009) Biochemistry, 48, pp. 4816-27. , 10. 1021/bi9002265. 19374450
Petock, J. M., Torshin, I. Y., Weber, I. T., Harrison, R. W., Analysis of protein structures reveals regions of rare backbone conformation at functional sites (2003) Proteins, 53, pp. 872-9. , 10. 1002/prot. 10484. 14635129
Cubellis, M. V., Caillez, F., Blundell, T. L., Lovell, S. C., Properties of polyproline II, a secondary structure element implicated in protein-protein interactions (2005) Proteins, 58, pp. 880-92. , 10. 1002/prot. 20327. 15657931
Worth, C. L., Bickerton, G. R., Schreyer, A., Forman, J. R., Cheng, T. M., Lee, S., Gong, S., Blundell, T. L., A structural bioinformatics approach to the analysis of nonsynonymous single nucleotide polymorphisms (nsSNPs) and their relation to disease (2007) J Bioinform Comput Biol, 5, pp. 1297-318. , 10. 1142/S0219720007003120. 18172930
Topham, C. M., Srinivasan, N., Blundell, T. L., Prediction of the stability of protein mutants based on structural environment-dependent amino acid substitution and propensity tables (1997) Protein Eng, 10, pp. 7-21. , 10. 1093/protein/10. 1. 7. 9051729
Gromiha, M. M., An, J., Kono, H., Oobatake, M., Uedaira, H., Prabakaran, P., Sarai, A., ProTherm, version 2. 0: Thermodynamic database for proteins and mutants (2000) Nucleic Acids Res, 28, pp. 283-5. , 10. 1093/nar/28. 1. 283. 10592247
http: //www. informedia. cs. cmu. edu/yanrong/MATLABArsenal/MATLABArsenal. htm, MATLAB-ArsenalAltschul, S. F., Madden, T. L., Schaffer, A. A., Zhang, J., Zhang, Z., Miller, W., Lipman, D. J., Gapped BLAST and PSI-BLAST: A new generation of protein database search programs (1997) Nucleic Acids Res, 25, pp. 3389-402. , 10. 1093/nar/25. 17. 3389. 9254694
Solis, M. A., Pascual, B., Bosca, M., Ramos, V., Carda, C., Monteagudo, C., Torregrosa, I., Miguel, A., New mutation in female patient with renal variant of Fabry disease and HIV J Nephrol, 23, pp. 231-3. , 20155722
Borglum, A. D., Byskov, A., Cubellis, M. V., Kruse, T. A., An EcoRI polymorphism for the PLAUR gene (1991) Nucleic Acids Res, 19, p. 6661. , 10. 1093/nar/19. 23. 6661-a. 1684424
http: //www. hgmd. cf. ac. uk/ac/index. php, HGMDMehta, A. B., Anderson-Fabry disease: Developments in diagnosis and treatment (2009) Int J Clin Pharmacol Ther, 47 (SUPPL. 1), pp. 1966-74. , 20040315
Smith, R. E., Lovell, S. C., Burke, D. F., Montalvao, R. W., Blundell, T. L., Andante: Reducing side-chain rotamer search space during comparative modeling using environment-specific substitution probabilities (2007) Bioinformatics, 23, pp. 1099-105. , 10. 1093/bioinformatics/btm073. 17341496
Connolly, M. L., Solvent-accessible surfaces of proteins and nucleic acids (1983) Science, 221, pp. 709-13. , 10. 1126/science. 6879170. 6879170
Orphanet Journal Of Rare Diseases (ISSN: 1750-1172), 2010 Dec 8; 5: 36-36.
Export to BibTeX Export to EndNote
Paper type: Abstract, Conference, Research Support, Non-U. S. Gov'T,
Impact factor: 5.933, 5-year impact factor: 5.968
Url: http://www.scopus.com/inward/record.url?eid=2-s2.0-78649755176&partnerID=40&md5=cca6f679bee731cc9815f901dba39d1e
Keywords: 1-Deoxynojirimycin Metabolism Pharmacology Therapeutic Use, Catalytic Domain, Fabry Disease Drug Therapy, Humans, Lysosomes Enzymology, Models, Molecular, Molecular Chaperones Metabolism Pharmacology Therapeutic Use, Mutation, Predictive Value Of Tests, Software, Structure-Activity Relationship, Treatment Outcome, Alpha-Galactosidase Chemistry Genetics Metabolism, Alpha Galactosidase, 1 Deoxynojirimycin, Article, Enzyme Activity, Enzyme Structure, Gene Mutation, Gene Sequence, Genetic Association, Missense Mutation, Thermodynamics, Thermostability, Chemical Structure, Computer Program, Enzyme Active Site, Structure Activity Relation,
Affiliations:
*** IBB - CNR ***
Istituto di Chimica Biomolecolare-CNR, Pozzuoli, Italy.
High Performance Computing and Networking Institute-CNR, Napoli, Italy
Dipartimento di Biologia Strutturale e Funzionale, Universita' Federico II, Napoli, Italy
Istituto di Biostrutture e Bioimmagini-CNR, Napoli, Italy
References:
Fan, J.Q., Ishii, S., Asano, N., Suzuki, Y., Accelerated transport and maturation of lysosomal alpha-galactosidase A in Fabry lymphoblasts by an enzyme inhibitor (1999) Nat Med, 5, pp. 112-5. , 10.1038/4801. 988384
Khanna, R., Benjamin, E.R., Pellegrino, L., Schilling, A., Rigat, B.A., Soska, R., Nafar, H., Lun, Y., The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of beta-glucosidase Febs J, 277, pp. 1618-38. , 10.1111/j.1742-4658.2010.07588.x. 20148966
Flanagan, J.J., Rossi, B., Tang, K., Wu, X., Mascioli, K., Donaudy, F., Tuzzi, M.R., Porto, C., The pharmacological chaperone 1-deoxynojirimycin increases the activity and lysosomal trafficking of multiple mutant forms of acid alpha-glucosidase (2009) Hum Mutat, 30, pp. 1683-92. , 10.1002/humu.21121. 19862843
Tropak, M.B., Reid, S.P., Guiral, M., Withers, S.G., Mahuran, D., Pharmacological enhancement of beta-hexosaminidase activity in fibroblasts from adult Tay-Sachs and Sandhoff Patients (2004) J Biol Chem, 279, pp. 13478-87. , 10.1074/jbc.M308523200. 14724290
Caciotti, A., Donati, M.A., D'Azzo, A., Salvioli, R., Guerrini, R., Zammarchi, E., Morrone, A., The potential action of galactose as a "chemical chaperone": Increase of beta galactosidase activity in fibroblasts from an adult GM1-gangliosidosis patient (2009) Eur J Paediatr Neurol, 13, pp. 160-4. , 10.1016/j.ejpn.2008.03.004. 18571950
Suzuki, Y., Chemical chaperone therapy for GM1-gangliosidosis (2008) Cell Mol Life Sci, 65, pp. 351-3. , 10.1007/s00018-008-7470-2. 18202827
Parenti, G., Treating lysosomal storage diseases with pharmacological chaperones: From concept to clinics (2009) EMBO Mol Med, 1, pp. 268-79. , 10.1002/emmm.200900036. 20049730
Beck, M., Therapy for lysosomal storage disorders IUBMB Life, 62, pp. 33-40. , 20014233
Loo, T.W., Clarke, D.M., Chemical and pharmacological chaperones as new therapeutic agents (2007) Expert Rev Mol Med, 9, pp. 1-18. , 10.1017/S1462399407000361. 17597553
Fan, J.Q., A counterintuitive approach to treat enzyme deficiencies: Use of enzyme inhibitors for restoring mutant enzyme activity (2008) Biol Chem, 389, pp. 1-11. , 10.1515/BC.2008.009. 18095864
Schaefer, E., Mehta, A., Gal, A., Genotype and phenotype in Fabry disease: Analysis of the Fabry Outcome Survey (2005) Acta Paediatr Suppl, 94, pp. 87-92. , discussion 79. 10.1080/08035320510031045. 15895718
Spada, M., Pagliardini, S., Yasuda, M., Tukel, T., Thiagarajan, G., Sakuraba, H., Ponzone, A., Desnick, R.J., High incidence of later-onset fabry disease revealed by newborn screening (2006) Am J Hum Genet, 79, pp. 31-40. , 10.1086/504601. 16773563
Benjamin, E.R., Flanagan, J.J., Schilling, A., Chang, H.H., Agarwal, L., Katz, E., Wu, X., Desnick, R.J., The pharmacological chaperone 1-deoxygalactonojirimycin increases alpha-galactosidase A levels in Fabry patient cell lines (2009) J Inherit Metab Dis, 32, pp. 424-40. , 10.1007/s10545-009-1077-0. 19387866
Shin, S.H., Kluepfel-Stahl, S., Cooney, A.M., Kaneski, C.R., Quirk, J.M., Schiffmann, R., Brady, R.O., Murray, G.J., Prediction of response of mutated alpha-galactosidase A to a pharmacological chaperone (2008) Pharmacogenet Genomics, 18, pp. 773-80. , 10.1097/FPC.0b013e32830500f4. 18698230
Shimotori, M., Maruyama, H., Nakamura, G., Suyama, T., Sakamoto, F., Itoh, M., Miyabayashi, S., Wataya-Kaneda, M., Novel mutations of the GLA gene in Japanese patients with Fabry disease and their functional characterization by active site specific chaperone (2008) Hum Mutat, 29, p. 331. , 10.1002/humu.9520. 18205205
Monserrat, L., Gimeno-Blanes, J.R., Marin, F., Hermida-Prieto, M., Garcia-Honrubia, A., Perez, I., Fernandez, X., Paya, E., Prevalence of fabry disease in a cohort of 508 unrelated patients with hypertrophic cardiomyopathy (2007) J Am Coll Cardiol, 50, pp. 2399-403. , 10.1016/j.jacc.2007.06.062. 18154965
Dundas, J., Ouyang, Z., Tseng, J., Binkowski, A., Turpaz, Y., Liang, J., CASTp: Computed atlas of surface topography of proteins with structural and topographical mapping of functionally annotated residues (2006) Nucleic Acids Res, 34, pp. 23116-8. , 10.1093/nar/gkl282. 16844972
http://sts.bioengr.uic.edu/castp/index.php, CASTPGarman, S.C., Garboczi, D.N., The molecular defect leading to Fabry disease: Structure of human alpha-galactosidase (2004) J Mol Biol, 337, pp. 319-35. , 10.1016/j.jmb.2004.01.035. 15003450
Guce, A.I., Clark, N.E., Salgado, E.N., Ivanen, D.R., Kulminskaya, A.A., Brumer, H., Garman, S.C., Catalytic mechanism of human alpha-galactosidase J Biol Chem, 285, pp. 3625-32. , 10.1074/jbc.M109.060145. 19940122
Lieberman, R.L., D'Aquino, J.A., Ringe, D., Petsko, G.A., Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability (2009) Biochemistry, 48, pp. 4816-27. , 10.1021/bi9002265. 19374450
Ishii, S., Yoshioka, H., Mannen, K., Kulkarni, A.B., Fan, J.Q., Transgenic mouse expressing human mutant alpha-galactosidase A in an endogenous enzyme deficient background: A biochemical animal model for studying active-site specific chaperone therapy for Fabry disease (2004) Biochim Biophys Acta, 1690, pp. 250-7. , 15511632
Petock, J.M., Torshin, I.Y., Weber, I.T., Harrison, R.W., Analysis of protein structures reveals regions of rare backbone conformation at functional sites (2003) Proteins, 53, pp. 872-9. , 10.1002/prot.10484. 14635129
Cubellis, M.V., Caillez, F., Blundell, T.L., Lovell, S.C., Properties of polyproline II, a secondary structure element implicated in protein-protein interactions (2005) Proteins, 58, pp. 880-92. , 10.1002/prot.20327. 15657931
Worth, C.L., Bickerton, G.R., Schreyer, A., Forman, J.R., Cheng, T.M., Lee, S., Gong, S., Blundell, T.L., A structural bioinformatics approach to the analysis of nonsynonymous single nucleotide polymorphisms (nsSNPs) and their relation to disease (2007) J Bioinform Comput Biol, 5, pp. 1297-318. , 10.1142/S0219720007003120. 18172930
Topham, C.M., Srinivasan, N., Blundell, T.L., Prediction of the stability of protein mutants based on structural environment-dependent amino acid substitution and propensity tables (1997) Protein Eng, 10, pp. 7-21. , 10.1093/protein/10.1.7. 9051729
Cheng, J., Randall, A., Baldi, P., Prediction of protein stability changes for single-site mutations using support vector machines (2006) Proteins, 62, pp. 1125-32. , 10.1002/prot.20810. 16372356
Gromiha, M.M., An, J., Kono, H., Oobatake, M., Uedaira, H., Prabakaran, P., Sarai, A., ProTherm, version 2.0: Thermodynamic database for proteins and mutants (2000) Nucleic Acids Res, 28, pp. 283-5. , 10.1093/nar/28.1.283. 10592247
http://www.informedia.cs.cmu.edu/yanrong/MATLABArsenal/MATLABArsenal.htm, MATLAB-ArsenalAltschul, S.F., Madden, T.L., Schaffer, A.A., Zhang, J., Zhang, Z., Miller, W., Lipman, D.J., Gapped BLAST and PSI-BLAST: A new generation of protein database search programs (1997) Nucleic Acids Res, 25, pp. 3389-402. , 10.1093/nar/25.17.3389. 9254694
Ramensky, V., Bork, P., Sunyaev, S., Human non-synonymous SNPs: Server and survey (2002) Nucleic Acids Res, 30, pp. 3894-900. , 10.1093/nar/gkf493. 12202775
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Prediction of the responsiveness to pharmacological chaperones: lysosomal human alpha-galactosidase, a case of study
Background: The pharmacological chaperones therapy is a promising approach to cure genetic diseases. It relies on substrate competitors used at sub-inhibitory concentration which can be administered orally, reach difficult tissues and have low cost. Clinical trials are currently carried out for Fabry disease, a lysosomal storage disorder caused by inherited genetic mutations of alpha-galactosidase. Regrettably, not all genotypes respond to these drugs. Results: We collected the experimental data available in literature on the enzymatic activity of ninety-six missense mutants of lysosomal alpha-galactosidase measured in the presence of pharmacological chaperones. We associated with each mutation seven features derived from the analysis of 3D-structure of the enzyme, two features associated with their thermo-dynamic stability and four features derived from sequence alone. Structural and thermodynamic analysis explains why some mutants of human lysosomal alpha-galactosidase cannot be rescued by pharmacological chaperones: approximately forty per cent of the non responsive cases examined can be correctly associated with a negative prognostic feature. They include mutations occurring in the active site pocket, mutations preventing disulphide bridge formation and severely destabilising mutations. Despite this finding, prediction of mutations responsive to pharmacological chaperones cannot be achieved with high accuracy relying on combinations of structure-and thermodynamic-derived features even with the aid of classical and state of the art statistical learning methods. We developed a procedure to predict responsive mutations with an accuracy as high as 87%: the method scores the mutations by using a suitable position-specific substitution matrix. Our approach is of general applicability since it does not require the knowledge of 3D-structure but relies only on the sequence. Conclusions: Responsiveness to pharmacological chaperones depends on the structural/functional features of the disease-associated protein, whose complex interplay is best reflected on sequence conservation by evolutionary pressure. We propose a predictive method which can be applied to screen novel mutations of alpha galactosidase. The same approach can be extended on a genomic scale to find candidates for therapy with pharmacological chaperones among proteins with unknown tertiary structures.
Background: The pharmacological chaperones therapy is a promising approach to cure genetic diseases. It relies on substrate competitors used at sub-inhibitory concentration which can be administered orally, reach difficult tissues and have low cost. Clinical trials are currently carried out for Fabry disease, a lysosomal storage disorder caused by inherited genetic mutations of alpha-galactosidase. Regrettably, not all genotypes respond to these drugs. Results: We collected the experimental data available in literature on the enzymatic activity of ninety-six missense mutants of lysosomal alpha-galactosidase measured in the presence of pharmacological chaperones. We associated with each mutation seven features derived from the analysis of 3D-structure of the enzyme, two features associated with their thermo-dynamic stability and four features derived from sequence alone. Structural and thermodynamic analysis explains why some mutants of human lysosomal alpha-galactosidase cannot be rescued by pharmacological chaperones: approximately forty per cent of the non responsive cases examined can be correctly associated with a negative prognostic feature. They include mutations occurring in the active site pocket, mutations preventing disulphide bridge formation and severely destabilising mutations. Despite this finding, prediction of mutations responsive to pharmacological chaperones cannot be achieved with high accuracy relying on combinations of structure-and thermodynamic-derived features even with the aid of classical and state of the art statistical learning methods. We developed a procedure to predict responsive mutations with an accuracy as high as 87%: the method scores the mutations by using a suitable position-specific substitution matrix. Our approach is of general applicability since it does not require the knowledge of 3D-structure but relies only on the sequence. Conclusions: Responsiveness to pharmacological chaperones depends on the structural/functional features of the disease-associated protein, whose complex interplay is best reflected on sequence conservation by evolutionary pressure. We propose a predictive method which can be applied to screen novel mutations of alpha galactosidase. The same approach can be extended on a genomic scale to find candidates for therapy with pharmacological chaperones among proteins with unknown tertiary structures.
Prediction of the responsiveness to pharmacological chaperones: lysosomal human alpha-galactosidase, a case of study
| ▼ Carbonic Anhydrase IX as target for the development of new anti-tumour drugs |
Prediction of the responsiveness to pharmacological chaperones: lysosomal human alpha-galactosidase, a case of study
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Tornatore L, Montil SM, Marasco D, Dathan N, Vitale RM, Benedetti E, Pedone C, Papa S, Franzoso G, Ruvo M
* Gadd45beta dimerization does not affect MKK7 binding (360 views)
Adv Exp Med Biol (ISSN: 0065-2598, 0065-2598print), 2009; 611: 367-368.
Impact Factor: 2.02
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Hilvo M, Baranauskiene L, Salzano AM, Scaloni A, Matulis D, Innocenti A, Scozzafava A, Monti SM, Di Fiore A, De Simone G, Lindfors M, Janis J, Valjakka J, Pastorekova S, Pastorek J, Kulomaa MS, Nordlund HR, Supuran CT, Parkkila S
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Jbc Papers (ISSN: 0021-9258, 1083-351x, 0021-9258linking), 2008 Oct 10; 283(41): 27799-27809.
Impact Factor: 5.52
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Grasso G, Fragai M, Rizzarelli E, Spoto G, Yeo KJ
* A new methodology for monitoring the activity of cdMMP-12 anchored and freeze-dried on Au (111) (357 views)
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Impact Factor: 3.664
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Ragno R, Simeoni S, Castellano S, Vicidomini C, Mai A, Caroli A, Tramontano A, Bonaccini C, Trojer P, Bauer I, Brosch G, Sbardella G
* Small molecule inhibitors of histone arginine methyltransferases: Homology modeling, molecular docking, binding mode analysis, and biological evaluations (938 views)
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Impact Factor: 4.895
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Cappiello M, Alterio V, Amodeo P, Del Corso A, Scaloni A, Pedone C, Moschini R, De Donatis GM, De Simone G, Mura U
* A new spot test for sulfhydryl-containing compounds (453 views)
Anal Biochem, 2006 Mar 14; 79(1-2): 610-611.
Impact Factor: 2.305
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Cappiello M, Alterio V, Amodeo P, Del Corso A, Scaloni A, Pedone C, Moschini R, De Donatis GM, De Simone G, Mura U
* Metal ion substitution in the catalytic site greatly affects the binding of sulfhydryl-containing compounds to leucyl aminopeptidase (769 views)
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Impact Factor: 3.633
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Grasso G, D'Agata R, Rizzarelli E, Spoto G, D'Andrea LD, Pedone C, Picardi A, Romanelli A, Fragai M, Yeo KJ
* Activity of anchored human matrix metalloproteinase-1 catalytic domain on Au(111) surfaces monitored by ESI-MS (577 views)
J Mass Spectrom (ISSN: 1076-5174, 1096-9888), 2005 Dec; 40(12): 1565-1571.
Impact Factor: 3.574
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Calhoun JR, Nastri F, Maglio O, Pavone V, Lombardi A, Degrado WF
* Artificial diiron proteins: From structure to function (490 views)
Biopolymers (ISSN: 0006-3525, 0006-6352, 0006-3525print), 2005; 80(2-3): 264-278.
Impact Factor: 2.545
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Falcigno L, Oliva R, D'Auria G, Maletta M, Dettin M, Pasquato A, Di Bello C, Paolillo L
* Structural investigation of the HIV-1 envelope glycoprotein gp160 cleavage site 3: Role of site-specific mutations (434 views)
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Impact Factor: 3.474
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Esposito L, Bruno I, Sica F, Raia CA, Giordano A, Rossi M, Mazzarella L, Zagari A
* Crystal structure of a ternary complex of the alcohol dehydrogenase from Sulfolobus solfataricus (545 views)
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Impact Factor: 3.922
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Oliva R, Falcigno L, D'Auria G, Dettin M, Scarinci C, Pasquato A, Di Bello C, Paolillo L
* Structural investigation of the HIV-1 envelope glycoprotein gp160 cleavage site (376 views)
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Impact Factor: 3.992
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Bashan A, Zarivach R, Schluenzen F, Agmon I, Harms J, Auerbach T, Baram D, Berisio R, Bartels H, Hansen HAS, Fucini P, Wilson D, Peretz M, Kessler M, Yonath A
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Impact Factor: 2.733
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Oliva R, Falcigno L, D'Auria G, Dettin M, Scarinci C, Pasquato A, Di Bello C, Paolillo L
* Structural investigation of the HIV-1 envelope glycoprotein gp160 cleavage site, 2: relevance of an N-terminal helix (360 views)
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Impact Factor: 2.92
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Bashan A, Agmon I, Zarivach R, Schluenzen F, Harms J, Berisio R, Bartels H, Franceschi F, Auerbach T, Hansen HAS, Kossoy E, Kessler M, Yonath A
* Structural basis of the ribosomal machinery for peptide bond formation, translocation, and nascent chain progression (465 views)
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Impact Factor: 16.835
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Merlino A, Vitagliano L, Ceruso M, Di Nola A, Mazzarella L
* Global and local motions in ribonuclease A: A molecular dynamics study (411 views)
Biopolymers (ISSN: 0006-3525, 0006-6352, 0006-3525print), 2002 Nov 15; 65(4): 274-283.
Impact Factor: 2.372
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Esposito L, Sica F, Raia CA, Giordano A, Rossi M, Mazzarella L, Zagari A
* Crystal Structure Of The Alcohol Dehydrogenase From The Hyperthermophilic Archaeon Sulfolobus Solfataricus At 1. 85 Angstrom Resolution (449 views)
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Impact Factor: 5.359
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Oliva R, Leone M, Falcigno L, D'Auria G, Dettin M, Scarinci C, Di Bello C, Paolillo L
* Structural investigation of the HIV-1 envelope glycoprotein gp160 cleavage site (499 views)
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Impact Factor: 4.238
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Berisio R, Sica F, Lamzin VS, Wilson KS, Zagari A, Mazzarella L
* Atomic resolution structures of ribonuclease A at six pH values (634 views)
Acta Crystallogr D Biol Crystallogr (ISSN: 0907-4449, 0907-4449linking), 2002; 58(3): 441-450.
Impact Factor: 1.76
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Bonomo RP, Boudet AM, Cozzolino R, Rizzarelli E, Santoro AM, Sterjiades R, Zappalà R
A comparative study of two isoforms of laccase secreted by the 'white- rot' fungus Rigidoporus lignosus, exhibiting significant structural and functional differences (308 views)
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Impact Factor: 1.162
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* The allosteric interplay between S-nitrosylation and glycine binding controls the activity of human serine racemase (70 views)
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Impact Factor: 4.739
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De Simone G, Bua S, Supuran CT, Alterio V
* Benzyl alcohol inhibits carbonic anhydrases by anchoring to the zinc coordinated water molecule (11 views)
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Impact Factor: 3.575
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Langella E, Buonanno M, De Simone G, Monti SM
* Intrinsically disordered features of carbonic anhydrase IX proteoglycan-like domain (30 views)
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Impact Factor: 9.261
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Romano M, Ruggiero A, Squeglia F, Maga G, Berisio R
* A Structural View of SARS-CoV-2 RNA Replication Machinery: RNA Synthesis, Proofreading and Final Capping (491 views)
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Impact Factor: 6.6
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Buemi MR, Di Fiore A, De Luca L, Angeli A, Mancuso F, Ferro S, Monti SM, Buonanno M, Russo E, De Sarro G, De Simone G, Supuran CT, Gitto R
* Exploring structural properties of potent human carbonic anhydrase inhibitors bearing a 4-(cycloalkylamino-1-carbonyl)benzenesulfonamide moiety (327 views)
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Impact Factor: 4.833
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Langella E, Buonanno M, Vullo D, Dathan N, Leone M, Supuran C, De Simone G, Monti SM
* Biochemical, biophysical and molecular dynamics studies on the proteoglycan-like domain of carbonic anhydrase IX (317 views)
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Impact Factor: 6.721
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Buonanno M, Di Fiore A, Langella E, D’ambrosio K, Supuran CT, Monti SM, De Simone G
* The Crystal Structure of a hCA VII Variant Provides Insights into the Molecular Determinants Responsible for Its Catalytic Behavior (304 views)
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Impact Factor: 3.687
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Magrì A, Tabbì G, Breglia R, De Gioia L, Fantucci P, Bruschi M, Bonomo RP, La Mendola D
* Copper ion interaction with the RNase catalytic site fragment of the angiogenin protein: an experimental and theoretical investigation (389 views) (PDF 179 views)
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Impact Factor: 4.099
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Ruggiero A, Squeglia F, Romano M, Vitagliano L, De Simone A, Berisio R
* Structure and dynamics of the multi-domain resuscitation promoting factor RpfB from Mycobacterium tuberculosis (359 views)
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Impact Factor: 3.107
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Maione V, Ruggiero A, Russo L, De Simone A, Pedone PV, Malgieri G, Berisio R, Isernia C
* NMR Structure and Dynamics of the Resuscitation Promoting Factor RpfC Catalytic Domain (355 views)
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Impact Factor: 3.057
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D'Ambrosio K, Carradori S, Monti SM, Buonanno M, Secci D, Vullo D, Supuran CT, De Simone G
* Out of the active site binding pocket for carbonic anhydrase inhibitors (481 views) (PDF 237 views)
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Impact Factor: 6.567
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Andreotti G, Citro V, Correra A, Cubellis MV
* A thermodynamic assay to test pharmacological chaperones for Fabry disease (396 views)
Bba-Gen Subjects (ISSN: 0925-4439, 0006-3002, 1570-9639), 2014 Mar; 1840(3): 1214-1224.
Impact Factor: 4.882
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D'Ambrosio K, Lopez M, Dathan NA, Ouahrani-bettache S, Köhler S, Ascione G, Monti SM, Winum JY, De Simone G
* Structural basis for the rational design of new anti-Brucella agents: The crystal structure of the C366S mutant of l-histidinol dehydrogenase from Brucella suis (454 views)
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Impact Factor: 2.963
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Vicari C, Saraiva IH, Maglio O, Nastri F, Pavone V, Louro RO, Lombardi A
* Artificial heme-proteins: determination of axial ligand orientations through paramagnetic NMR shifts (327 views)
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Impact Factor: 6.834
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Lin L-L, Chen Y-Y, Chi M-C, Merlino A
* Low resolution X-ray structure of γ-glutamyltranspeptidase from Bacillus licheniformis: Opened active site cleft and a cluster of acid residues potentially involved in the recognition of a metal ion (511 views)
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Impact Factor: 4.882
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Correale S, Ruggiero A, Capparelli R, Pedone E, Berisio R
* Structures of free and inhibited forms of the L, D-transpeptidase LdtMt1 from Mycobacterium tuberculosis (402 views)
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Impact Factor: 7.232
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Cammisa M, Correra A, Andreotti G, Cubellis MV
* Fabry_CEP: a tool to identify Fabry mutations responsive to pharmacological chaperones (415 views)
Orphanet Journal Of Rare Diseases (ISSN: 1750-1172), 2013 Jul 24; 8: 111-111.
Impact Factor: 3.958
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Squeglia F, Romano M, Ruggiero A, Vitagliano L, De Simone A, Berisio R
* Carbohydrate recognition by RpfB from mycobacterium tuberculosis unveiled by crystallographic and molecular dynamics analyses (505 views)
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Impact Factor: 3.832
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Cammisa M, Correra A, Andreotti G, Cubellis MV
* Identification and analysis of conserved pockets on protein surfaces (1329 views)
Bmc Bioinformatics (ISSN: 1471-2105), 2013 Apr 22; 14: S9-S9.
Impact Factor: 2.672
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Ruggiero A, Marchant J, Squeglia F, Makarov V, De Simone A, Berisio R
* Molecular determinants of inactivation of the resuscitation promoting factor B from Mycobacterium tuberculosis (507 views)
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Impact Factor: 2.983
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Doti N, Scognamiglio PL, Madonna S, Scarponi C, Ruvo M, Perretta G, Albanesi C, Marasco D
* New mimetic peptides of the kinase-inhibitory region (KIR) of SOCS1 through focused peptide libraries (1200 views)
Biochemical Journal, 2012 Apr 1; 443: 231-240.
Impact Factor: 4.779
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Di Fiore A, Maresca A, Supuran CT, De Simone G
* Hydroxamate represents a versatile zinc binding group for the development of new carbonic anhydrase inhibitors (361 views)
Chem Commun (ISSN: 1359-7345, 1364-548x, 1364-548xelectronic), 2012; 48(70): 8838-8840.
Impact Factor: 6.378
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Folgiero V, Di Carlo SE, Bossi G, Spugnini E, Di Benedetto A, Bon G, Milella M, Fabi A, Accardo A, Morelli G, Mottolese M, Falcioni R
* Inhibition of p85, the non-catalytic subunit of phosphatidylinositol 3-kinase, exerts potent antitumor activity in human breast cancer cells (518 views)
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Impact Factor: 6.044
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Cantisani M, Vitiello M, Falanga A, Finamore E, Galdiero M, Galdiero S
* Peptides complementary to the active loop of porin P2 from Haemophilus influenzae modulate its activity (527 views)
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Impact Factor: 3.463
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Andreotti G, Citro V, De Crescenzo A, Orlando P, Cammisa M, Correra A, Cubellis MV
* Therapy of Fabry disease with pharmacological chaperones: from in silico predictions to in vitro tests (1698 views)
Orphanet Journal Of Rare Diseases (ISSN: 1750-1172), 2011 Oct 17; 6: 66-66.
Impact Factor: 5.074
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Merlino A, Russo Krauss I, Castellano I, De Vendittis E, Rossi B, Conte M, Vergara A, Sica F
* Structure and flexibility in cold-adapted iron superoxide dismutases: The case of the enzyme isolated from Pseudoalteromonas haloplanktis (543 views)
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Impact Factor: 3.5
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Pedone E, Limauro D, D'Ambrosio K, De Simone G, Bartolucci S
* Multiple catalytically active thioredoxin folds: a winning strategy for many functions (755 views)
Cell Mol Life Sci Cellular And Molecular Life Sciences (ISSN: 1420-682x), 2010 Nov; 67(22): 3797-3814.
Impact Factor: 7.047
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Alterio V, Monti SM, Truppo E, Pedone C, Supuran CT, De Simone G
* The first example of a significant active site conformational rearrangement in a carbonic anhydrase-inhibitor adduct: The carbonic anhydrase I-topiramate complex (447 views)
Org Biomol Chem (ISSN: 1477-0520, 1477-0539, 1477-0539electronic), 2010 Sep 7; 8(15): 3528-3533.
Impact Factor: 3.451
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Langella E, Pierre S, Ghattas W, Giorgi M, Réglier M, Saviano M, Esposito L, Hardré R
* Probing The Peptidylglycine Alpha-Hydroxylating Monooxygenase Active Site With Novel 4-Phenyl-3-Butenoic Acid Based Inhibitors (422 views)
Chemmedchem (ISSN: 1860-7179, 1860-7187, 1860-7187electronic), 2010 Sep 3; 5(9): 1568-1576.
Impact Factor: 3.306
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Alterio V, Aurilia V, Romanelli A, Parracino A, Saviano M, D'Auria S, De Simone G
* Crystal structure of an S-formylglutathione hydrolase from pseudoalteromonas haloplanktis TAC125 (372 views)
Biopolymers (ISSN: 0006-3525, 0006-6352, 0006-3525print), 2010 Sep; 93(8): 669-677.
Impact Factor: 2.572
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Di Fiore A, Monti SM, Innocenti A, Winum J-Y, De Simone G, Supuran CT
* Carbonic anhydrase inhibitors: Crystallographic and solution binding studies for the interaction of a boron-containing aromatic sulfamide with mammalian isoforms I-XV (352 views)
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Impact Factor: 2.661
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Faiella M, Andreozzi C, de Rosales RT, Pavone V, Maglio O, Nastri F, DeGrado WF, Lombardi A
* An artificial di-iron oxo-protein with phenol oxidase activity (433 views)
Nat Chem Biol (ISSN: 1552-4450), 2009 Dec; 5(12): 882-884.
Impact Factor: 16.058
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Merlino A, Russo Krauss I, Perillo M, Mattia CA, Ercole C, Picone D, Vergara A, Sica F
* Toward an Antitumor Form of Bovine Pancreatic Ribonuclease: The Crystal Structure of Three Noncovalent Dimeric Mutants (399 views)
Biopolymers (ISSN: 0006-3525, 0006-6352, 0006-3525print), 2009 Dec; 91(12): 1029-1037.
Impact Factor: 2.605
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Vitale RM, Alterio V, Innocenti A, Winum JY, Monti SM, De Simone G, Supuran CT
* Carbonic anhydrase inhibitors. Comparison of aliphatic sulfamate/bis- sulfamate adducts with isozymes II and IX as a platform for designing tight-binding, more isoform-selective inhibitors (480 views)
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Impact Factor: 4.802
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Alterio V, Hilvo M, Di Fiore A, Supuran CT, Pan P, Parkkila S, Scaloni A, Pastorek J, Pastorekova S, Pedone C, Scozzafava A, Monti SM, De Simone G
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P Natl Acad Sci Usa (ISSN: 0027-8424, 1091-6490, 0027-8424print), 2009 Sep 22; 106(38): 16233-16238.
Impact Factor: 9.432
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Pennacchio A, Esposito L, Zagari A, Rossi M, Raia CA
* Role of Tryptophan 95 in substrate specificity and structural stability of Sulfolobus solfataricus alcohol dehydrogenase (298 views)
Extremophiles (ISSN: 1431-0651, 1433-4909, 1433-4909electronic), 2009 Sep; 13(5): 751-761.
Impact Factor: 2
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Ruggiero A, Tizzano B, Pedone E, Pedone C, Wilmanns M, Berisio R
* Crystal Structure of the Resuscitation-Promoting Factor (Delta DUF)RpfB from M. tuberculosis (588 views)
J Mol Biol (ISSN: 0022-2836, 1089-8638, 1089-8638electronic), 2009 Jan 9; 385(1): 153-162.
Impact Factor: 3.871
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Palmieri G, Catara G, Saviano M, Langella E, Gogliettino M, Rossi M
* First archaeal PEPB-serine protease inhibitor from sulfolobus solfataricus with noncanonical amino acid sequence in the reactive-site loop (397 views)
J Proteome Res (ISSN: 1535-3893), 2009 Jan; 8(1): 327-334.
Impact Factor: 5.132
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Limauro D, Saviano M, Galdi I, Rossi M, Bartolucci S, Pedone E
* Sulfolobus solfataricus protein disulphide oxidoreductase: insight into the roles of its redox sites (236 views)
Protein Engineering Design & Selection (ISSN: 1741-0126), 2009 Jan; 22(1): 19-26.
Impact Factor: 2.596
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Tornatore L, Montil SM, Marasco D, Dathan N, Vitale RM, Benedetti E, Pedone C, Papa S, Franzoso G, Ruvo M
* Gadd45beta dimerization does not affect MKK7 binding (360 views)
Adv Exp Med Biol (ISSN: 0065-2598, 0065-2598print), 2009; 611: 367-368.
Impact Factor: 2.02
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Hilvo M, Baranauskiene L, Salzano AM, Scaloni A, Matulis D, Innocenti A, Scozzafava A, Monti SM, Di Fiore A, De Simone G, Lindfors M, Janis J, Valjakka J, Pastorekova S, Pastorek J, Kulomaa MS, Nordlund HR, Supuran CT, Parkkila S
* Biochemical characterization of CA IX, one of the most active carbonic anhydrase isozymes (460 views)
Jbc Papers (ISSN: 0021-9258, 1083-351x, 0021-9258linking), 2008 Oct 10; 283(41): 27799-27809.
Impact Factor: 5.52
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Grasso G, Fragai M, Rizzarelli E, Spoto G, Yeo KJ
* A new methodology for monitoring the activity of cdMMP-12 anchored and freeze-dried on Au (111) (357 views)
Journal Of The American Society For Mass Spectrometry (ISSN: 1044-0305), 2007 May; 18(5): 961-969.
Impact Factor: 3.664
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Ragno R, Simeoni S, Castellano S, Vicidomini C, Mai A, Caroli A, Tramontano A, Bonaccini C, Trojer P, Bauer I, Brosch G, Sbardella G
* Small molecule inhibitors of histone arginine methyltransferases: Homology modeling, molecular docking, binding mode analysis, and biological evaluations (938 views)
J Med Chem (ISSN: 0022-2623, 1520-4804, 0022-2623print), 2007 Mar 22; 50(6): 1241-1253.
Impact Factor: 4.895
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Cappiello M, Alterio V, Amodeo P, Del Corso A, Scaloni A, Pedone C, Moschini R, De Donatis GM, De Simone G, Mura U
* A new spot test for sulfhydryl-containing compounds (453 views)
Anal Biochem, 2006 Mar 14; 79(1-2): 610-611.
Impact Factor: 2.305
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Cappiello M, Alterio V, Amodeo P, Del Corso A, Scaloni A, Pedone C, Moschini R, De Donatis GM, De Simone G, Mura U
* Metal ion substitution in the catalytic site greatly affects the binding of sulfhydryl-containing compounds to leucyl aminopeptidase (769 views)
Biochemistry (ISSN: 0006-2960, 1520-4995, 1520-4995electronic), 2006 Mar 14; 45(10): 3226-3234.
Impact Factor: 3.633
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Grasso G, D'Agata R, Rizzarelli E, Spoto G, D'Andrea LD, Pedone C, Picardi A, Romanelli A, Fragai M, Yeo KJ
* Activity of anchored human matrix metalloproteinase-1 catalytic domain on Au(111) surfaces monitored by ESI-MS (577 views)
J Mass Spectrom (ISSN: 1076-5174, 1096-9888), 2005 Dec; 40(12): 1565-1571.
Impact Factor: 3.574
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Calhoun JR, Nastri F, Maglio O, Pavone V, Lombardi A, Degrado WF
* Artificial diiron proteins: From structure to function (490 views)
Biopolymers (ISSN: 0006-3525, 0006-6352, 0006-3525print), 2005; 80(2-3): 264-278.
Impact Factor: 2.545
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Falcigno L, Oliva R, D'Auria G, Maletta M, Dettin M, Pasquato A, Di Bello C, Paolillo L
* Structural investigation of the HIV-1 envelope glycoprotein gp160 cleavage site 3: Role of site-specific mutations (434 views)
Chembiochem (ISSN: 1439-4227, 1439-7633, 1439-7633electronic), 2004 Dec 3; 5(12): 1653-1661.
Impact Factor: 3.474
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Esposito L, Bruno I, Sica F, Raia CA, Giordano A, Rossi M, Mazzarella L, Zagari A
* Crystal structure of a ternary complex of the alcohol dehydrogenase from Sulfolobus solfataricus (545 views)
Biochemistry (ISSN: 0006-2960, 1520-4995, 1520-4995electronic), 2003 Dec 16; 42(49): 14397-14407.
Impact Factor: 3.922
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Oliva R, Falcigno L, D'Auria G, Dettin M, Scarinci C, Pasquato A, Di Bello C, Paolillo L
* Structural investigation of the HIV-1 envelope glycoprotein gp160 cleavage site (376 views)
Chembiochem (ISSN: 1439-4227, 1439-7633, 1439-7633electronic), 2003 Sep 4; 4(8): 727-733.
Impact Factor: 3.992
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Bashan A, Zarivach R, Schluenzen F, Agmon I, Harms J, Auerbach T, Baram D, Berisio R, Bartels H, Hansen HAS, Fucini P, Wilson D, Peretz M, Kessler M, Yonath A
* Ribosomal crystallography: Peptide bond formation and its inhibition (588 views)
Biopolymers (ISSN: 0006-3525, 0006-6352, 0006-3525print), 2003 Sep; 70(1): 19-41.
Impact Factor: 2.733
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Oliva R, Falcigno L, D'Auria G, Dettin M, Scarinci C, Pasquato A, Di Bello C, Paolillo L
* Structural investigation of the HIV-1 envelope glycoprotein gp160 cleavage site, 2: relevance of an N-terminal helix (360 views)
Chembiochem (ISSN: 1439-4227, 1439-7633, 1439-7633electronic), 2003 Aug 4; 4(8): 727-733.
Impact Factor: 2.92
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Bashan A, Agmon I, Zarivach R, Schluenzen F, Harms J, Berisio R, Bartels H, Franceschi F, Auerbach T, Hansen HAS, Kossoy E, Kessler M, Yonath A
* Structural basis of the ribosomal machinery for peptide bond formation, translocation, and nascent chain progression (465 views)
Mol Cell (ISSN: 1097-2765, 1097-4164), 2003 Jan; 11(1): 91-102.
Impact Factor: 16.835
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Merlino A, Vitagliano L, Ceruso M, Di Nola A, Mazzarella L
* Global and local motions in ribonuclease A: A molecular dynamics study (411 views)
Biopolymers (ISSN: 0006-3525, 0006-6352, 0006-3525print), 2002 Nov 15; 65(4): 274-283.
Impact Factor: 2.372
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Esposito L, Sica F, Raia CA, Giordano A, Rossi M, Mazzarella L, Zagari A
* Crystal Structure Of The Alcohol Dehydrogenase From The Hyperthermophilic Archaeon Sulfolobus Solfataricus At 1. 85 Angstrom Resolution (449 views)
J Mol Biol (ISSN: 0022-2836, 1089-8638, 1089-8638electronic), 2002 Apr 26; 318(2): 463-477.
Impact Factor: 5.359
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Oliva R, Leone M, Falcigno L, D'Auria G, Dettin M, Scarinci C, Di Bello C, Paolillo L
* Structural investigation of the HIV-1 envelope glycoprotein gp160 cleavage site (499 views)
Chemistry (ISSN: 0947-6539, 1521-3765, 1521-3765electronic), 2002 Mar 15; 8(6): 1467-1473.
Impact Factor: 4.238
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Berisio R, Sica F, Lamzin VS, Wilson KS, Zagari A, Mazzarella L
* Atomic resolution structures of ribonuclease A at six pH values (634 views)
Acta Crystallogr D Biol Crystallogr (ISSN: 0907-4449, 0907-4449linking), 2002; 58(3): 441-450.
Impact Factor: 1.76
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Bonomo RP, Boudet AM, Cozzolino R, Rizzarelli E, Santoro AM, Sterjiades R, Zappalà R
A comparative study of two isoforms of laccase secreted by the 'white- rot' fungus Rigidoporus lignosus, exhibiting significant structural and functional differences (308 views)
J Chem Res (ISSN: 0162-0134, 1873-3344, 0162-0134print), 1998 Sep; 71(3-4): 205-211.
Impact Factor: 1.162
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58 Records (54 excluding Abstracts).
Total impact factor: 268.178 (250.43 excluding Abstracts).
Total 5 year impact factor: 275.732 (254.857 excluding Abstracts).
Total impact factor: 268.178 (250.43 excluding Abstracts).
Total 5 year impact factor: 275.732 (254.857 excluding Abstracts).
493 views. Last view on Monday 22 May 2023, 9:53:51
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