2g7i

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{{STRUCTURE_2g7i| PDB=2g7i | SCENE= }}
{{STRUCTURE_2g7i| PDB=2g7i | SCENE= }}
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'''Structure of Human Complement Factor H Carboxyl Terminal Domains 19-20: a Basis for Atypical Hemolytic Uremic Syndrome'''
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===Structure of Human Complement Factor H Carboxyl Terminal Domains 19-20: a Basis for Atypical Hemolytic Uremic Syndrome===
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==Overview==
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Factor H (FH) is the key regulator of the alternative pathway of complement. The carboxyl-terminal domains 19-20 of FH interact with the major opsonin C3b, glycosaminoglycans, and endothelial cells. Mutations within this area are associated with atypical haemolytic uremic syndrome (aHUS), a disease characterized by damage to endothelial cells, erythrocytes, and kidney glomeruli. The structure of recombinant FH19-20, solved at 1.8 A by X-ray crystallography, reveals that the short consensus repeat domain 20 contains, unusually, a short alpha-helix, and a patch of basic residues at its base. Most aHUS-associated mutations either destabilize the structure or cluster in a unique region on the surface of FH20. This region is close to, but distinct from, the primary heparin-binding patch of basic residues. By mutating five residues in this region, we show that it is involved, not in heparin, but in C3b binding. Therefore, the majority of the aHUS-associated mutations on the surface of FH19-20 interfere with the interaction between FH and C3b. This obviously leads to impaired control of complement attack on plasma-exposed cell surfaces in aHUS.
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(as it appears on PubMed at http://www.pubmed.gov), where 16601698 is the PubMed ID number.
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{{ABSTRACT_PUBMED_16601698}}
==About this Structure==
==About this Structure==
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[[Category: Immune system]]
[[Category: Immune system]]
[[Category: Regulator]]
[[Category: Regulator]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun May 4 04:46:50 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Jul 27 18:56:26 2008''

Revision as of 15:56, 27 July 2008

Image:2g7i.png

Template:STRUCTURE 2g7i

Structure of Human Complement Factor H Carboxyl Terminal Domains 19-20: a Basis for Atypical Hemolytic Uremic Syndrome

Template:ABSTRACT PUBMED 16601698

About this Structure

2G7I is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Structure of complement factor H carboxyl-terminus reveals molecular basis of atypical haemolytic uremic syndrome., Jokiranta TS, Jaakola VP, Lehtinen MJ, Parepalo M, Meri S, Goldman A, EMBO J. 2006 Apr 19;25(8):1784-94. Epub 2006 Apr 6. PMID:16601698

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