ImmunoDeficiency Resource (IDR)

Names

HUGO name: C5

Alias(es):

Localization

Reference sequences

Chromosomal location

Maps

Variations / Mutations

• C5base; Mutation registry for C5 deficiency.

Other gene-based resources

Description

Protein function:

Activation of C5 by a C5 convertase initiates the spontaneous assembly of the late complement components, C5-C9, into the Membrane Attack Complex (MAC). C5b has a transient binding site for C6. The C5b-C6 complex is the foundation upon which the lytic complex is assembled.

Subunit:

C5 precursor is first processed by the removal of 4 basic residues, forming two chains, beta & alpha, linked by a disulfide bond. C5 convertase activates C5 by cleaving the alpha chain, releasing C5a anaphylatoxin & generating C5b (beta chain + alpha' chain).

Caution:

Ref.3 sequence differs from that shown from position 855 onward due to the presence of an Alu repeat.

Structures (PDB)

1KJS	NMR Solution Structure Of C5A At pH 5.2, 303K, 20 Structures
1CFA	Solution Structure Of A Semi-Synthetic C5A Receptor Antagonist At pH 5.2, 303K, NMR, 20 Structures

Domains

Anaphylatoxin-like domain: 698-732

Other features

Signal peptide: 1-18

Complement c5 beta chain: 19-673

Propeptide: 674-677

Complement c5 alpha chain: 678-1676

Released active peptide C5a anaphylatoxin: 678-751

C5b (alpha'): 752-1676

Disulfide bond: 698-724

Disulfide bond: 699-731

Disulfide bond: 711-732

N-linked (glcnac...) glycosylation site: 741

N-linked (glcnac...) glycosylation site: 911

N-linked (glcnac...) glycosylation site: 1115

N-linked (glcnac...) glycosylation site: 1630

Other related resourses:

Expression pattern for human

Tissue	Expression (%)	Clones
corresponding non cancerous liver tissue	27.80	16:13909
germ cell, yolk sac	21.40	1:1129
lung epithelial cells tissue nos 359-368	5.53	1:4366
bone marrow stroma	4.53	1:5331
human skeletal muscle	4.50	2:10746
liver	3.71	4:26031
hypothalamus	3.68	1:6565
hepatocellular carcinoma	3.40	2:14226
spleen	3.34	1:7229
kidney, pooled	3.26	1:7404