常规说明ab94098 is a 293T cell transfected lysate in which Human C1s has been transiently over-expressed using a pCMV-C1s plasmid. The lysate is provided in 1X Sample Buffer.
Note: For more details about how the transfected lysate was prepared view preparation notes
背景Function: C1s B chain is a serine protease that combines with C1q and C1s 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.
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.
PTM: The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.