信號(hào)通路: Complement and coagulation cascades;Prion diseases;Systemic lupus erythematosus;
功能: disease:Defects in C8B are a cause of complement C8 deficiency type II [MIM:120960]. Patients with deficiency of C8 suffer from recurrent bacterial infections, predominantly from Neisseria meningitidis.,function:C8 is a constituent of the membrane attack complex. C8 binds to the C5b-7 complex, forming the C5b-8 complex. C5-b8 binds C9 and acts as a catalyst in the polymerization of C9.,online information:C8B mutation db,polymorphism:The sequence shown is that of allotype C8B B.,PTM:N-glycosylated; contains one or two bound glycans. Not O-glycosylated.,similarity:Belongs to the complement C6/C7/C8/C9 family.,similarity:Contains 1 EGF-like domain.,similarity:Contains 1 LDL-receptor class A domain.,similarity:Contains 1 MACPF domain.,similarity:Contains 2 TSP type-1 domains.,subunit:C8 is composed of three chains: alpha, beta and gamma. The beta chain binds to the C8 alpha chain and to the C5b-C7 complex, presumably to C5b. It is essential to the incorporation of C8 into the C5b-C8 complex.,
信號(hào)通路: Complement and coagulation cascades;Prion diseases;Systemic lupus erythematosus;
功能: disease:Defects in C8B are a cause of complement C8 deficiency type II [MIM:120960]. Patients with deficiency of C8 suffer from recurrent bacterial infections, predominantly from Neisseria meningitidis.,function:C8 is a constituent of the membrane attack complex. C8 binds to the C5b-7 complex, forming the C5b-8 complex. C5-b8 binds C9 and acts as a catalyst in the polymerization of C9.,online information:C8B mutation db,polymorphism:The sequence shown is that of allotype C8B B.,PTM:N-glycosylated; contains one or two bound glycans. Not O-glycosylated.,similarity:Belongs to the complement C6/C7/C8/C9 family.,similarity:Contains 1 EGF-like domain.,similarity:Contains 1 LDL-receptor class A domain.,similarity:Contains 1 MACPF domain.,similarity:Contains 2 TSP type-1 domains.,subunit:C8 is composed of three chains: alpha, beta and gamma. The beta chain binds to the C8 alpha chain and to the C5b-C7 complex, presumably to C5b. It is essential to the incorporation of C8 into the C5b-C8 complex.,
相關(guān)產(chǎn)品: RS0001,RS0002,YM3028,YM3029
細(xì)胞定位: Secreted.
組織表達(dá): Liver,Plasma,
科研貨號(hào): PLA019396
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