其他名称： C4A; CO4; CPAMD2; Complement C4-A; Acidic complement C4; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 2; C4B; CO4; CPAMD3; Complement C4-B; Basic complement C4; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 3
实测条带： 180kD
信号通路： Complement and coagulation cascades;Systemic lupus erythematosus;
功能： disease:Defects in C4A are the cause of C4A deficiency [MIM:120810].,disease:The C4A6 allotype is totally deficient in hemolytic activity.,function:C4 plays a central role in the activation of the classical pathway of the complement system. It is processed by activated C1 which removes from the alpha chain the C4a anaphylatoxin. The remaining alpha chain fragment C4b is the major activation product and is an essential subunit of the C3 convertase (C4b2a) and the C5 convertase (C3bC4b2a) enzymes of the classical complement pathway.,function:Derived from proteolytic degradation of complement C4, C4a anaphylatoxin is a mediator of local inflammatory process. It induces the contraction of smooth muscle, increases vascular permeability and causes histamine release from mast cells and basophilic leukocytes.,miscellaneous:C4A allotypes react more rapidly with the amino group of peptide antigens while C4B allotypes react more rapidly with the hydroxyl group of carbohydrate antigens.,online information:Blood group antigen gene mutation database,polymorphism:Human complement component C4 is polymorphic at two loci, C4A and C4B. 13 alleles of C4A and 22 alleles of C4B have been detected. The allele shown here is C4A4. The C4A alleles carry the Rodgers (Rg) while the C4B alleles carry the Chido (Ch) blood group antigens.,PTM:Prior to secretion, the single-chain precursor is enzymatically cleaved to yield the non-identical chains (alpha, beta and gamma). During activation, the alpha chain is cleaved by C1 into C4a and C4b, and C4b stays linked to the beta and gamma chains. Further degradation of C4b by C1 into the inactive fragments C4c and C4d blocks the generation of C3 convertase.,similarity:Contains 1 anaphylatoxin-like domain.,similarity:Contains 1 NTR domain.,subunit:Circulates in blood as a disulfide-linked trimer of an alpha, beta and gamma chain.,
组织表达： Complement component C4 is expressed at highest levels in the liver, at moderate levels in the adrenal cortex, adrenal medulla, thyroid gland, and the kidney, and at lowest levels in the heart, ovary, small intestine, thymus, pancreas and spleen. The extra-hepatic sites of expression may be important for the local protection and inflammatory response.
其他名称： C4A; CO4; CPAMD2; Complement C4-A; Acidic complement C4; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 2; C4B; CO4; CPAMD3; Complement C4-B; Basic complement C4; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 3
实测条带： 180kD
信号通路： Complement and coagulation cascades;Systemic lupus erythematosus;
功能： disease:Defects in C4A are the cause of C4A deficiency [MIM:120810].,disease:The C4A6 allotype is totally deficient in hemolytic activity.,function:C4 plays a central role in the activation of the classical pathway of the complement system. It is processed by activated C1 which removes from the alpha chain the C4a anaphylatoxin. The remaining alpha chain fragment C4b is the major activation product and is an essential subunit of the C3 convertase (C4b2a) and the C5 convertase (C3bC4b2a) enzymes of the classical complement pathway.,function:Derived from proteolytic degradation of complement C4, C4a anaphylatoxin is a mediator of local inflammatory process. It induces the contraction of smooth muscle, increases vascular permeability and causes histamine release from mast cells and basophilic leukocytes.,miscellaneous:C4A allotypes react more rapidly with the amino group of peptide antigens while C4B allotypes react more rapidly with the hydroxyl group of carbohydrate antigens.,online information:Blood group antigen gene mutation database,polymorphism:Human complement component C4 is polymorphic at two loci, C4A and C4B. 13 alleles of C4A and 22 alleles of C4B have been detected. The allele shown here is C4A4. The C4A alleles carry the Rodgers (Rg) while the C4B alleles carry the Chido (Ch) blood group antigens.,PTM:Prior to secretion, the single-chain precursor is enzymatically cleaved to yield the non-identical chains (alpha, beta and gamma). During activation, the alpha chain is cleaved by C1 into C4a and C4b, and C4b stays linked to the beta and gamma chains. Further degradation of C4b by C1 into the inactive fragments C4c and C4d blocks the generation of C3 convertase.,similarity:Contains 1 anaphylatoxin-like domain.,similarity:Contains 1 NTR domain.,subunit:Circulates in blood as a disulfide-linked trimer of an alpha, beta and gamma chain.,
组织表达： Complement component C4 is expressed at highest levels in the liver, at moderate levels in the adrenal cortex, adrenal medulla, thyroid gland, and the kidney, and at lowest levels in the heart, ovary, small intestine, thymus, pancreas and spleen. The extra-hepatic sites of expression may be important for the local protection and inflammatory response.
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