4j1y

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'''Unreleased structure'''
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{{STRUCTURE_4j1y| PDB=4j1y | SCENE= }}
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===The X-ray crystal structure of human complement protease C1s zymogen===
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The entry 4j1y is ON HOLD until Paper Publication
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==Disease==
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[[http://www.uniprot.org/uniprot/C1S_HUMAN C1S_HUMAN]] Defects in C1S are the cause of complement component C1s deficiency (C1SD) [MIM:[http://omim.org/entry/613783 613783]]. A rare defect resulting in C1 deficiency and impaired activation of the complement classical pathway. C1 deficiency generally leads to severe immune complex disease with features of systemic lupus erythematosus and glomerulonephritis.
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Authors: Perry, A.J., Wilmann, P.M., Wijeyewickrema, L.C., Irving, J.A., Duncan, R.C., Pang, S.S., Pike, R.N., Whisstock, J.C.
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==Function==
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[[http://www.uniprot.org/uniprot/C1S_HUMAN C1S_HUMAN]] C1s B chain is a serine protease that combines with C1q and C1r to form C1, the first component of the classical pathway of the complement system. C1r activates C1s so that it can, in turn, activate C2 and C4.
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Description: The X-ray crystal structure of human complement protease C1s zymogen
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==About this Structure==
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[[4j1y]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4J1Y OCA].
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[[Category: Complement subcomponent C1s]]
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[[Category: Homo sapiens]]
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[[Category: Duncan, R C.]]
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[[Category: Irving, J A.]]
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[[Category: Pang, S S.]]
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[[Category: Perry, A J.]]
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[[Category: Pike, R N.]]
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[[Category: Whisstock, J C.]]
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[[Category: Wijeyewickrema, L C.]]
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[[Category: Wilmann, P M.]]
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[[Category: C2]]
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[[Category: C4]]
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[[Category: Extracellular]]
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[[Category: Hydrolase]]
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[[Category: Hydrolysis]]

Revision as of 11:31, 24 April 2013

Template:STRUCTURE 4j1y

Contents

The X-ray crystal structure of human complement protease C1s zymogen

Disease

[C1S_HUMAN] Defects in C1S are the cause of complement component C1s deficiency (C1SD) [MIM:613783]. A rare defect resulting in C1 deficiency and impaired activation of the complement classical pathway. C1 deficiency generally leads to severe immune complex disease with features of systemic lupus erythematosus and glomerulonephritis.

Function

[C1S_HUMAN] C1s B chain is a serine protease that combines with C1q and C1r to form C1, the first component of the classical pathway of the complement system. C1r activates C1s so that it can, in turn, activate C2 and C4.

About this Structure

4j1y is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Proteopedia Page Contributors and Editors (what is this?)

OCA

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