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Rabbit Anti-C1s /FITC Conjugated antibody
background:
The complement component proteins, C1, C3, C4, and C5, are potent anaphylatoxins that are released during complement activation. Binding of these proteins to their respective G protein-coupled receptors induces proinflammatory events, such as cellular degranulation, smooth muscle contraction, arachidonic acid metabolism, cytokine release, leukocyte activation, and cellular chemotaxis. C1q, together with proenzymes C1r and C1s, yield C1, the first component of the classical pathway of the serum complement system. C1 consists of a calcium dependent trimolecular complex of C1r, C1s and C1q in a 2:2:1 ratio. Activated C1s is in the form of a disulfide-linked heterodimer consisting of a heavy chain and a light chain. Defects in the gene encoding for C1s can cause selective C1s deficiency, a disorder characterized by early onset of various autoimmune diseases.
Function:
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.
Subunit:
C1 is a calcium-dependent trimolecular complex of C1q, C1r and C1s in the molar ration of 1:2:2. Activated C1s is an disulfide-linked heterodimer of a heavy chain and a light chain.
Post-translational modifications:
The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.
DISEASE:
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.
Similarity:
Belongs to the peptidase S1 family.
Contains 2 CUB domains.
Contains 1 EGF-like domain.
Contains 1 peptidase S1 domain.
Contains 2 Sushi (CCP/SCR) domains.
Database links:
Entrez Gene: 716 Human
Entrez Gene: 50908 Mouse
Entrez Gene: 192262 Rat
Omim: 120580 Human
SwissProt: P09871 Human
SwissProt: Q8CG14 Mouse
SwissProt: Q6P6T1 Rat
Unigene: 458355 Human
Unigene: 4037 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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