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CD59; CD59 glycoprotein; membrane attack complex inhibition factor; MACIF; MAC-inhibitory protein; MAC-IP; protectin; MEM43 antigen; membrane inhibitor of reactive lysis; MIRL; 20 kD homologous restriction factor; HRF-20; HRF20; 1F5 antigen (MIC11, MIN1, MIN2, MIN3, MSK21)
Function:
- associates with/activates lck [1]
- regulates complement mediated cell lysis by inhibiting formation of membrane attack complex (MAC)
- expression on erythrocytes important for survival
- acts by binding to the C8 &/or C9 complements of the assembling MAC, thus preventing incorporation of the multiple copies of C9 required for complete formation of the osmolytic pore
- this inhibitor appears to be species-specific
- involved in signal transduction for T-cell activation complexed to a protein tyrosine kinase
- interacts with T-cell surface antigen CD2
Structure:
- N- & O-glycosylated
- the N-glycosylation mainly consists of a family of biantennary complex-type structures with & without lactosamine extensions & outer arm fucose residues
- also significant amounts of triantennary complexes (22%)
- variable sialylation also present in the Asn-43 oligosaccharide
- predominant O-glycans are mono-sialylated forms of the disaccharide, gal-beta-1,3galNAc; their sites of attachment are probably on Thr-76 & Thr-77
- the GPI-anchor of soluble urinary CD59 has no inositol-associated phospholipid, but is composed of seven different GPI-anchor variants of one or more monosaccharide units
- major variants contain sialic acid, mannose & glucosamine
- contains 1 UPAR/Ly6 domain
Compartment:
- cell membrane; lipid-anchor, GPI-anchor
- secreted, soluble form found in a number of tissues
Expression:
- expressed in most cells
Pathology:
- defects, if together with CD55 defects, associated with paroxysmal nocturnal hemoglobinuria
- glycation is found in diabetic subjects, but only at minimal levels in nondiabetic subjects
- glycated CD59 lacks MAC-inhibitory function & confers to vascular complications of diabetes
Notes:
- the soluble form from urine retains its specific complement binding activity, but exhibits greatly diminished ability to inhibit MAC assembly on cell membranes
Related
lck proto-oncogene protein
General
cluster-of-differentiation antigen; cluster designation antigen; CD antigen
glypiated protein
Properties
SIZE: entity length = 128 aa
MW = 14 kD
COMPARTMENT: plasma membrane
CELL-REGION: apical region
CELL: epithelial cell
MOTIF: signal sequence {1-25}
UPAR/Ly6 {26-108}
MOTIF: cysteine residue {C28}
MODIFICATION: cysteine residue {C51}
cysteine residue {C31}
MODIFICATION: cysteine residue {C38}
cysteine residue {C38}
MODIFICATION: cysteine residue {C31}
N-glycosylation site {N43}
cysteine residue {C44}
MODIFICATION: cysteine residue {C64}
cysteine residue {C51}
MODIFICATION: cysteine residue {C28}
cysteine residue {C64}
MODIFICATION: cysteine residue {C44}
cysteine residue {C70}
MODIFICATION: cysteine residue {C88}
Thr glycosylation site {T76}
Thr glycosylation site {T77}
cysteine residue {C88}
MODIFICATION: cysteine residue {C70}
cysteine residue {C89}
MODIFICATION: cysteine residue {C94}
cysteine residue {C94}
MODIFICATION: cysteine residue {C89}
glycosyl phosphatidylinositol [GPI] membrane anchor {N102}
Database Correlations
OMIM 107271
UniProt P13987
Pfam PF00021
Entrez Gene 966
Kegg hsa:966
References
- Bolen JB, Rowley RB, Spana C, Tsygankov AY.
The Src family of tyrosine protein kinases in hemopoietic
signal transduction.
FASEB J. 1992 Dec;6(15):3403-9. Review.
PMID: 1281458
- UniProt :accession P13987
- http://www.pathologyoutlines.com/cdmarkers.html
15 October 2002
- Entrez Gene :accession 966
- CD59base; Note: CD59 mutation db
http://bioinf.uta.fi/CD59base/