Structural determinants in prion protein folding and stability (1549 views)
J Mol Biol (ISSN: 0022-2836, 1089-8638, 0022-2836linking), 2014 Nov 11; 426(22): 3796-3810.
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Paper type: Journal Article, Research Support, Non-U. S. Gov'T,
Impact factor: 4.333, 5-year impact factor: 3.702
Url: http://www.scopus.com/inward/record.url?eid=2-s2.0-84908192029&partnerID=40&md5=958c192b126bdb6ba8d6c9e76cc7b65d
Keywords: Disulfide Bond, Energy Landscape, Intermediate State, N-Terminal Domain, Prion-Susceptible Species, Octapeptide, Prion Protein, Article, Dielectric Constant, Enthalpy, Human, Nonhuman, Protein Analysis, Protein Folding, Protein Function, Protein Stability, Protein Structure, Simulation, Animals, Calorimetry, Differential Scanning, Disulfides Chemistry Metabolism, Molecular Dynamics Simulation, Mutation, Prions Chemistry Genetics Metabolism, Protein Isoforms, Protein Processing, Post-Translational, Spectrometry, Fluorescence,
Affiliations:
*** IBB - CNR ***
Department of Neuroscience, Scuola Internazionale Superiore di Studi Avanzati, Via Bonomea 265Trieste, Italy
Italian Institute of Technology, Scuola Internazionale Superiore di Studi Avanzati Unit, Via Bonomea 265Trieste, Italy
Department of Physics, Scuola Internazionale Superiore di Studi AvanzatiTrieste, Italy
National Research Council, Institute of Biostructures and Bioimaging, Viale Andrea Doria 6Catania, Italy
Elettra - Sincrotrone Trieste S.C.p.A., AREA Science ParkBasovizza Trieste, Italy
European Center for the Sustainable Impact of Nanotechnology, Veneto Nanotech S.C.p.A.Rovigo, Italy
Laboratory of Biochemistry, Institut Químic de Sarrià, Universitat Ramon Llull, Via Augusta 390Barcelona, Spain
References:
Not available.
J Mol Biol (ISSN: 0022-2836, 1089-8638, 0022-2836linking), 2014 Nov 11; 426(22): 3796-3810.
Export to BibTeX Export to EndNote
Paper type: Journal Article, Research Support, Non-U. S. Gov'T,
Impact factor: 4.333, 5-year impact factor: 3.702
Url: http://www.scopus.com/inward/record.url?eid=2-s2.0-84908192029&partnerID=40&md5=958c192b126bdb6ba8d6c9e76cc7b65d
Keywords: Disulfide Bond, Energy Landscape, Intermediate State, N-Terminal Domain, Prion-Susceptible Species, Octapeptide, Prion Protein, Article, Dielectric Constant, Enthalpy, Human, Nonhuman, Protein Analysis, Protein Folding, Protein Function, Protein Stability, Protein Structure, Simulation, Animals, Calorimetry, Differential Scanning, Disulfides Chemistry Metabolism, Molecular Dynamics Simulation, Mutation, Prions Chemistry Genetics Metabolism, Protein Isoforms, Protein Processing, Post-Translational, Spectrometry, Fluorescence,
Affiliations:
*** IBB - CNR ***
Department of Neuroscience, Scuola Internazionale Superiore di Studi Avanzati, Via Bonomea 265Trieste, Italy
Italian Institute of Technology, Scuola Internazionale Superiore di Studi Avanzati Unit, Via Bonomea 265Trieste, Italy
Department of Physics, Scuola Internazionale Superiore di Studi AvanzatiTrieste, Italy
National Research Council, Institute of Biostructures and Bioimaging, Viale Andrea Doria 6Catania, Italy
Elettra - Sincrotrone Trieste S.C.p.A., AREA Science ParkBasovizza Trieste, Italy
European Center for the Sustainable Impact of Nanotechnology, Veneto Nanotech S.C.p.A.Rovigo, Italy
Laboratory of Biochemistry, Institut Químic de Sarrià, Universitat Ramon Llull, Via Augusta 390Barcelona, Spain
References:
Not available.
Structural determinants in prion protein folding and stability
Prions are responsible for a heterogeneous group of fatal neurodegenerative diseases, involving post-translational modifications of the cellular prion protein. Epidemiological studies on Creutzfeldt-Jakob disease, a prototype prion disorder, show a majority of cases being sporadic, while the remaining occurrences are either genetic or iatrogenic. The molecular mechanisms by which PrPC is converted into its pathological isoform have not yet been established. While point mutations and seeds trigger the protein to cross the energy barriers, thus causing genetic and infectious transmissible spongiform encephalopathies, respectively, the mechanism responsible for sporadic forms remains unclear. Since prion diseases are protein-misfolding disorders, we investigated prion protein folding and stability as functions of different milieus. Using spectroscopic techniques and atomistic simulations, we dissected the contribution of major structural determinants, also defining the energy landscape of prion protein. In particular, we elucidated (i) the essential role of the octapeptide region in prion protein folding and stability, (ii) the presence of a very enthalpically stable intermediate in prion-susceptible species, and (iii) the role of the disulfide bridge in prion protein folding.
Prions are responsible for a heterogeneous group of fatal neurodegenerative diseases, involving post-translational modifications of the cellular prion protein. Epidemiological studies on Creutzfeldt-Jakob disease, a prototype prion disorder, show a majority of cases being sporadic, while the remaining occurrences are either genetic or iatrogenic. The molecular mechanisms by which PrPC is converted into its pathological isoform have not yet been established. While point mutations and seeds trigger the protein to cross the energy barriers, thus causing genetic and infectious transmissible spongiform encephalopathies, respectively, the mechanism responsible for sporadic forms remains unclear. Since prion diseases are protein-misfolding disorders, we investigated prion protein folding and stability as functions of different milieus. Using spectroscopic techniques and atomistic simulations, we dissected the contribution of major structural determinants, also defining the energy landscape of prion protein. In particular, we elucidated (i) the essential role of the octapeptide region in prion protein folding and stability, (ii) the presence of a very enthalpically stable intermediate in prion-susceptible species, and (iii) the role of the disulfide bridge in prion protein folding.
Structural determinants in prion protein folding and stability
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Structural determinants in prion protein folding and stability
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View Export to BibTeX Export to EndNote
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Impact Factor: 4.881
View Export to BibTeX Export to EndNote
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Impact Factor: 2.876
View Export to BibTeX Export to EndNote
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Impact Factor: 3.196
View Export to BibTeX Export to EndNote
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J Pept Sci (ISSN: 1075-2617, 1099-1387, 1075-2617linking), 2015; 21(5): 415-425.
Impact Factor: 1.951
View Export to BibTeX Export to EndNote
Limauro D, De Simone G, Pirone L, Bartolucci S, D'Ambrosio K, Pedone E
* Sulfolobus solfataricus thiol redox puzzle: characterization of an atypical protein disulfide oxidoreductase (801 views)
Extremophiles (ISSN: 1431-0651, 1433-4909, 1433-4909electronic), 2014 Mar; 18(2): 219-228.
Impact Factor: 2.306
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Petruk AA, Varriale S, Coscia MR, Mazzarella L, Merlino A, Oreste U
* The Structure Of The Cd3 Zeta Zeta Transmembrane Dimer In Popc And Raft-Like Lipid Bilayer: A Molecular Dynamics Study (807 views)
Bba-Gen Subjects (ISSN: 0005-2736, 0006-3002e, 0005-2736print), 2013 Nov; 1828(11): 2637-2645.
Impact Factor: 3.498
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Cammisa M, Correra A, Andreotti G, Cubellis MV
* Fabry_CEP: a tool to identify Fabry mutations responsive to pharmacological chaperones (845 views)
Orphanet Journal Of Rare Diseases (ISSN: 1750-1172), 2013 Jul 24; 8: 111-111.
Impact Factor: 3.958
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Palmieri M, Malgieri G, Russo L, Baglivo I, Esposito S, Netti F, Del Gatto A, De Paola I, Zaccaro L, Pedone PV, Isernia C, Milardi D, Fattorusso R
* Structural Zn(II) Implies a Switch from Fully Cooperative to Partly Downhill Folding in Highly Homologous Proteins (595 views)
J Am Chem Soc (ISSN: 0002-7863, 0002-2786, 1520-5126), 2013 Apr 2; 135(13): 5220-5228.
Impact Factor: 11.444
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Avitabile C, Capparelli R, Rigano MM, Fulgione A, Barone A, Pedone C, Romanelli A
* Antimicrobial peptides from plants: Stabilization of the γ core of a tomato defensin by intramolecular disulfide bond (1270 views)
J Pept Sci (ISSN: 1075-2617, 1099-1387, 1075-2617print), 2013 Apr; 19(4): 240-245.
Impact Factor: 1.862
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Poletto M, Vascotto C, Scognamiglio PL, Lirussi L, Marasco D, Tell G
* Role Of The Unstructured N-Terminal Domain Of The Human Apurinic/Apyrimidinic Endonuclease 1 (hape1) In The Modulation Of Its Interaction With Nucleic Acids And Nucleophosmin (npm1) (855 views)
Biochem J (ISSN: 0264-6021, 1470-8728electronic, 0264-6021linking), 2013; 452(3): 545-557.
Impact Factor: 4.779
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Travaglia A, La Mendola D, Magrì A, Nicoletti VG, Pietropaolo A, Rizzarelli E
* Copper, BDNF and Its N-terminal domain: inorganic features and biological perspectives (795 views)
Chemistry (ISSN: 1521-3765, 0947-6539, 1521-3765electronic), 2012 Dec 3; 18(49): 15618-15631.
Impact Factor: 5.831
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Pica A, Russo Krauss I, Castellano I, Rossi M, La Cara F, Graziano G, Sica F, Merlino A
* Exploring The Unfolding Mechanism Of Gamma-Glutamyltranspeptidases: The Case Of The Thermophilic Enzyme From Geobacillus Thermodenitrificans (1628 views)
Bba-Gen Subjects (ISSN: 1570-9639, 0006-3002, 0925-4439), 2012 Apr; 1824(4): 571-577.
Impact Factor: 3.733
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Esposito L, Ruggiero A, Masullo M, Ruocco MR, Lamberti A, Arcari P, Zagari A, Vitagliano L
* Crystallographic and spectroscopic characterizations of Sulfolobus solfataricus TrxA1 provide insights into the determinants of thioredoxin fold stability (680 views)
J Struct Biol (ISSN: 1047-8477, 1047-8477linking), 2012 Feb; 177(2): 506-512.
Impact Factor: 3.361
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Benetti F, Biarnes X, Attanasio F, Rizzarelli E, Laio A, Legname G
* Description and Characterization of a Highly Stable Enthalpic Intermediate State in Mouse Prion Protein Folding (496 views)
Prion (ISSN: 1933-6896), 2011 Apr; 5: N/D-N/D.
Impact Factor: 2.85
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Travaglia A, Arena G, Fattorusso R, Isernia C, La Mendola D, Malgieri G, Nicoletti VG, Rizzarelli E
* The inorganic perspective of nerve growth factor: interactions of Cu2+ and Zn2+ with the N-terminus fragment of nerve growth factor encompassing the recognition domain of the TrkA receptor (845 views)
Chemistry (ISSN: 1521-3765, 0947-6539, 1521-3765electronic), 2011 Mar 21; 17(13): 3726-3738.
Impact Factor: 5.925
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Barile E, Leone M, Barile E, Dahl R, Pellecchia M
* Design and NMR Studies of Cyclic Peptides Targeting the N-Terminal Domain of the Protein Tyrosine Phosphatase YopH (2470 views)
Chem Biol Drug Des (ISSN: 1747-0277, 1747-0285), 2011 Jan; 77(1): 12-19.
Impact Factor: 2.282
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Fantini D, Vascotto C, Marasco D, D'Ambrosio C, Romanello M, Vitagliano L, Pedone C, Poletto M, Cesaratto L, Quadrifoglio F, Scaloni A, Radicella JP, Tell G
* Critical lysine residues within the overlooked N-terminal domain of human APE1 regulate its biological functions (807 views)
Nucleic Acids Res (ISSN: 1362-4962, 0305-1048, 1362-4962electronic), 2010 Dec; 38(22): 8239-8256.
Impact Factor: 7.836
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Di Natale G, Pappalardo G, Milardi D, Sciacca MF, Attanasio F, La Mendola D, Rizzarelli E
* Membrane interactions and conformational preferences of human and avian prion N-terminal tandem repeats: The role of copper(II) ions, pH, and membrane mimicking environments (813 views)
J Phys Chem B (ISSN: 1520-6106, 1520-5207, 1520-5207electronic), 2010 Nov 4; 114(43): 13830-13838.
Impact Factor: 3.603
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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 (1226 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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Limauro D, D'Ambrosio K, Langella E, De Simone G, Galdi I, Pedone C, Pedone E, Bartolucci S
* Exploring the catalytic mechanism of the first dimeric Bcp: Functional, structural and docking analyses of Bcp4 from Sulfolobus solfataricus (736 views)
Biochimie (ISSN: 1638-6183, 0300-9084, 1638-6183electronic), 2010 Oct; 92(10): 1435-1444.
Impact Factor: 3.787
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La Mendola D, Magrì A, Campagna T, Campitiello MA, Raiola L, Isernia C, Hansson O, Bonomo RP, Rizzarelli E
* A doppel alpha-helix peptide fragment mimics the copper(II) interactions with the whole protein (1761 views)
Chemistry (ISSN: 0947-6539, 1521-3765, 1521-3765electronic), 2010 Jun 1; 16(21): 6212-6223.
Impact Factor: 5.476
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De Simone G, Supuran CT
* Carbonic anhydrase IX: Biochemical and crystallographic characterization of a novel antitumor target (808 views)
Bba-Gen Subjects (ISSN: 1570-9639, 0006-3002, 0925-4439), 2010 Feb; 1804(2): 404-409.
Impact Factor: 2.773
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Pedone E, Limauro D, Bartolucci S
* The Machinery for Oxidative Protein Folding in Thermophiles (vol 10, pg 157, 2008) (756 views)
Antioxid Redox Signal (ISSN: 1523-0864, 1557-7716, 1523-0864linking), 2010 Jan; 12(1): 157-169.
Impact Factor: 8.209
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Ruggiero A, Masullo M, Marasco D, Ruocco MR, Grimaldi P, Arcari P, Zagari A, Vitagliano L
* The dimeric structure of Sulfolobus solfataricus thioredoxin A2 and the basis of its thermostability (1042 views)
Proteins (ISSN: 0887-3585, 1097-0134, 1097-0134electronic), 2009 Dec; 77(4): 1004-1008.
Impact Factor: 3.085
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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
* Crystal structure of the catalytic domain of the tumor-associated human carbonic anhydrase IX (899 views)
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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D'Ambrosio K, Limauro D, Pedone E, Galdi I, Pedone C, Bartolucci S, De Simone G
* Insights into the catalytic mechanism of the Bcp family: functional and structural analysis of Bcp1 from Sulfolobus solfataricus (879 views)
Proteins (ISSN: 1097-0134, 0887-3585, 1097-0134electronic), 2009 Sep; 76(4): 995-1006.
Impact Factor: 3.085
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Accardo A, Tesauro D, Morisco A, Mangiapia G, Vaccaro M, Gianolio E, Heenan RK, Paduano L, Morelli G
* Micelles obtained by aggregation of gemini surfactants containing the CCK8 peptide and a gadolinium complex (932 views)
J Biol Inorg Chem (ISSN: 0949-8257, 1432-1327electronic, 0949-8257linking), 2009 May; 14(4): 587-599.
Impact Factor: 3.415
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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 (1124 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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Dalla Serra M, Cirioni O, Vitale RM, Renzone G, Coraiola M, Giacometti A, Potrich C, Baroni E, Guella G, Sanseverino M, De Luca S, Scalise G, Amodeo P, Scaloni A
* Structural features of distinctin affecting peptide biological and biochemical properties (994 views)
Biochemistry (ISSN: 0006-2960, 1520-4995, 1520-4995electronic), 2008 Jul 29; 47(30): 7888-7899.
Impact Factor: 3.379
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Pedone E, Limauro D, Bartolucci S
* The machinery for oxidative protein folding in thermophiles (663 views)
Antioxid Redox Signal (ISSN: 1523-0864, 1557-7716, 1523-0864linking), 2008 Jan; 10(1): 157-169.
Impact Factor: 6.19
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Pedone E, Limauro D, D'Alterio R, Rossi M, Bartolucci S
* Characterization of a multifunctional protein disulfide oxidoreductase from Sulfolobus solfataricus (648 views)
Febs Journal (ISSN: 1742-464x), 2006 Dec; 273(23): 5407-5420.
Impact Factor: 3.033
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De Simone A, Berisio R, Zagari A, Vitagliano L
* Limited Tendency Of Alpha-Helical Residues To Form Disulfide Bridges: A Structural Explanation (931 views)
J Pept Sci (ISSN: 1075-2617, 1099-1387, 1075-2617print), 2006 Dec; 12(12): 740-747.
Impact Factor: 1.801
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Calvanese L, Saporito A, Marasco D, D'Auria G, Minchiotti G, Pedone C, Paolillo L, Falcigno L, Ruvo M
* Solution structure of mouse Cripto CFC domain and its inactive variant Trp107Ala (1018 views)
J Med Chem (ISSN: 0022-2623, 1520-4804, 0022-2623print), 2006 Nov 30; 49(24): 7054-7062.
Impact Factor: 5.115
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D'Ambrosio K, Pedone E, Langella E, De Simone G, Rossi M, Pedone C, Bartolucci S
* A novel member of the protein disulfide oxidoreductase family from Aeropyrum pernix K1: Structure, function and electrostatics (983 views)
J Mol Biol (ISSN: 0022-2836, 1089-8638, 1089-8638electronic), 2006 Sep 29; 362(4): 743-752.
Impact Factor: 4.89
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De Simone G, Vitale RM, Di Fiore A, Pedone C, Scozzafava A, Montero JL, Winum JY, Supuran CT
* Carbonic anhydrase inhibitors: Hypoxia-activatable sulfonamides incorporating disulfide bonds that target the tumor-associated isoform IX (872 views)
J Med Chem (ISSN: 0022-2623, 1520-4804, 0022-2623print), 2006 Sep 7; 49(18): 5544-5551.
Impact Factor: 5.115
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Pedone E, D'Ambrosio K, De Simone G, Rossi M, Pedone C, Bartolucci S
* Insights on a new PDI-like family: Structural and functional analysis of a protein disulfide oxidoreductase from the bacterium Aquifex aeolicus (793 views)
J Mol Biol (ISSN: 0022-2836, 1089-8638, 1089-8638electronic), 2006 Feb 10; 356(1): 155-164.
Impact Factor: 4.89
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Merlino A, Mazzarella L, Carannante A, Di Fiore A, Di Donato A, Notomista E, Sica F
* The importance of dynamic effects on the enzyme activity: X-ray structure and molecular dynamics of onconase mutants (983 views)
Jbc Papers (ISSN: 0021-9258, 1083-351x, 0021-9258linking), 2005 May 6; 280(18): 17953-17960.
Impact Factor: 5.854
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Picone D, Di Fiore A, Ercole C, Franzese M, Sica F, Tomaselli S, Mazzarella L
* The role of the hinge loop in domain swapping - The special case of bovine seminal ribonuclease (861 views)
Jbc Papers (ISSN: 0021-9258, 1083-351x, 0021-9258linking), 2005 Apr 8; 280(14): 13771-13778.
Impact Factor: 5.854
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Esposito L, De Simone A, Zagari A, Vitagliano L
* Correlation Between Omega And Psi Dihedral Angles In Protein Structures (1531 views)
J Mol Biol (ISSN: 0022-2836, 1089-8638, 1089-8638electronic), 2005 Apr 1; 347(3): 483-487.
Impact Factor: 5.229
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Sica F, Di Fiore A, Merlino A, Mazzarella L
* Structure and stability of the non-covalent swapped dimer of bovine seminal ribonuclease - An enzyme tailored to evade ribonuclease protein inhibitor (859 views)
Jbc Papers (ISSN: 0021-9258, 1083-351x, 0021-9258linking), 2004 Sep 27; 279(35): 36753-36760.
Impact Factor: 6.355
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Merlino A, Vitagliano L, Sica F, Zagari A, Mazzarella L
* Population shift vs induced fit: The case of bovine seminal ribonuclease swapping dimer (594 views)
Biopolymers (ISSN: 0006-3525, 0006-6352, 0006-3525print), 2004 Apr 15; 73(6): 689-695.
Impact Factor: 2.863
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Sica F, Di Fiore A, Zagari A, Mazzarella L
* The unswapped chain of bovine seminal ribonuclease: Crystal structure of the free and liganded monomeric derivative (532 views)
Proteins (ISSN: 0887-3585, 1097-0134, 1097-0134electronic), 2003 Sep 1; 52(2): 263-271.
Impact Factor: 4.313
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Orru S, Vitagliano L, Esposito L, Mazzarella L, Marino G, Ruoppolo M
Effect of deamidation on folding of ribonuclease (997 views)
Protein Sci (ISSN: 0961-8368, 1469-896xelectronic), 2000 Dec; 9(12): 2577-2582.
Impact Factor: 3.869
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45 Records (43 excluding Abstracts).
Total impact factor: 204.666 (197.858 excluding Abstracts).
Total 5 year impact factor: 196.96 (188.597 excluding Abstracts).
Total impact factor: 204.666 (197.858 excluding Abstracts).
Total 5 year impact factor: 196.96 (188.597 excluding Abstracts).
1549 views. Last view on Wednesday 16 April 2025, 5:58:45
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