C8 α-polypeptide deficiency

Gene Information

Names

HUGO name: C8A

Alias(es):

  • complement component 8, alpha polypeptide
  • Complement component C8 alpha chain precursor

Localization

Reference sequences

Chromosomal location

Maps

Protein Information

Description

Protein function:

C8 is a constituent of the membrane attack complex (MAC). 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.

Subunit:

C8 is composed of three chains: alpha, beta and gamma. The alpha and gamma chains are disulfide bonded.

Subcellular location:

Secreted.

Structures (PDB)

Domains

Tsp type-1 1 domain: 38-93

    Ldl-receptor class a domain: 94-132

      Egf-like domain: 493-529

        Tsp type-1 2 domain: 536-584

          Other features

          Signal peptide: 1-20

            Propeptide: 21-30

              Complement component c8 alpha chain: 31-584

                Disulfide bond: 96-108

                  Disulfide bond: 102-121

                    Disulfide bond: 115-130

                      Disulfide bond interchain (with c8-gamma): 194

                        Disulfide bond: 375-399

                          Other related resourses:

                          Expression

                          Expression pattern for human

                          Tissue Expression (%) Clones
                          gall bladder 52.46 3:2435
                          hepatocellular carcinoma 17.96 6:14226
                          liver 11.45 7:26031
                          corresponding non cancerous liver tissue 9.18 3:13909
                          spleen 5.89 1:7229
                          pool, liver+spleen 2.78 4:61327
                          lung 0.27 1:155782

                          Animal models

                          Fly

                          C. elegans