1lyy
From Proteopedia
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==Overview== | ==Overview== | ||
| - | Tissue deposition of soluble proteins as amyloid fibrils underlies a range | + | Tissue deposition of soluble proteins as amyloid fibrils underlies a range of fatal diseases. The two naturally occurring human lysozyme variants are both amyloidogenic, and are shown here to be unstable. They aggregate to form amyloid fibrils with transformation of the mainly helical native fold, observed in crystal structures, to the amyloid fibril cross-beta fold. Biophysical studies suggest that partly folded intermediates are involved in fibrillogenesis, and this may be relevant to amyloidosis generally. |
==Disease== | ==Disease== | ||
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[[Category: Lysozyme]] | [[Category: Lysozyme]] | ||
[[Category: Single protein]] | [[Category: Single protein]] | ||
| - | [[Category: Blake, C | + | [[Category: Blake, C C.F.]] |
[[Category: Sunde, M.]] | [[Category: Sunde, M.]] | ||
[[Category: 4-glycan-hydrolase]] | [[Category: 4-glycan-hydrolase]] | ||
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[[Category: hydrolase]] | [[Category: hydrolase]] | ||
| - | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 13:49:50 2008'' |
Revision as of 11:49, 21 February 2008
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AMYLOIDOGENIC VARIANT (ASP67HIS) OF HUMAN LYSOZYME
Contents |
Overview
Tissue deposition of soluble proteins as amyloid fibrils underlies a range of fatal diseases. The two naturally occurring human lysozyme variants are both amyloidogenic, and are shown here to be unstable. They aggregate to form amyloid fibrils with transformation of the mainly helical native fold, observed in crystal structures, to the amyloid fibril cross-beta fold. Biophysical studies suggest that partly folded intermediates are involved in fibrillogenesis, and this may be relevant to amyloidosis generally.
Disease
Known diseases associated with this structure: Amyloidosis, renal OMIM:[153450], Microphthalmia, syndromic 1 OMIM:[309800]
About this Structure
1LYY is a Single protein structure of sequence from Homo sapiens. Active as Lysozyme, with EC number 3.2.1.17 Full crystallographic information is available from OCA.
Reference
Instability, unfolding and aggregation of human lysozyme variants underlying amyloid fibrillogenesis., Booth DR, Sunde M, Bellotti V, Robinson CV, Hutchinson WL, Fraser PE, Hawkins PN, Dobson CM, Radford SE, Blake CC, Pepys MB, Nature. 1997 Feb 27;385(6619):787-93. PMID:9039909
Page seeded by OCA on Thu Feb 21 13:49:50 2008
