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Protein

CD59 glycoprotein

Gene

CD59

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Potent inhibitor of the complement membrane attack complex (MAC) action. Acts by binding to the C8 and/or C9 complements of the assembling MAC, thereby 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.
The soluble form from urine retains its specific complement binding activity, but exhibits greatly reduced ability to inhibit MAC assembly on cell membranes.

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Enzyme and pathway databases

ReactomeiR-HSA-204005. COPII (Coat Protein 2) Mediated Vesicle Transport.
R-HSA-5694530. Cargo concentration in the ER.
R-HSA-6807878. COPI-mediated anterograde transport.
R-HSA-977606. Regulation of Complement cascade.

Names & Taxonomyi

Protein namesi
Recommended name:
CD59 glycoprotein
Alternative name(s):
1F5 antigen
20 kDa homologous restriction factor
Short name:
HRF-20
Short name:
HRF20
MAC-inhibitory protein
Short name:
MAC-IP
MEM43 antigen
Membrane attack complex inhibition factor
Short name:
MACIF
Membrane inhibitor of reactive lysis
Short name:
MIRL
Protectin
CD_antigen: CD59
Gene namesi
Name:CD59
Synonyms:MIC11, MIN1, MIN2, MIN3, MSK21
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 11

Organism-specific databases

HGNCiHGNC:1689. CD59.

Subcellular locationi

GO - Cellular componenti

  • anchored component of external side of plasma membrane Source: MGI
  • cell surface Source: UniProtKB
  • compact myelin Source: Ensembl
  • endoplasmic reticulum-Golgi intermediate compartment membrane Source: Reactome
  • endoplasmic reticulum membrane Source: Reactome
  • ER to Golgi transport vesicle membrane Source: Reactome
  • extracellular exosome Source: UniProtKB
  • extracellular space Source: UniProtKB
  • focal adhesion Source: UniProtKB
  • Golgi membrane Source: Reactome
  • membrane Source: ProtInc
  • plasma membrane Source: Reactome
  • sarcolemma Source: Ensembl
  • vesicle Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Cell membrane, Membrane, Secreted

Pathology & Biotechi

Involvement in diseasei

Hemolytic anemia, CD59-mediated, with or without polyneuropathy (HACD59)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal recessive disorder characterized by infantile onset of chronic hemolysis and a relapsing-remitting polyneuropathy, often exacerbated by infection, and manifested as hypotonia, limb muscle weakness, and hyporeflexia.
See also OMIM:612300
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti89 – 891C → Y in HACD59. 1 Publication
Corresponds to variant rs397514767 [ dbSNP | Ensembl ].
VAR_070124

Mutagenesis

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Mutagenesisi29 – 291Y → R: No loss of function. 1 Publication
Mutagenesisi33 – 331N → R or Q: No loss of function. 1 Publication
Mutagenesisi37 – 371D → R: No loss of function. 1 Publication
Mutagenesisi48 – 481F → R: Some loss of function. Some lysis. 1 Publication
Mutagenesisi49 – 491D → R: Loss of function. Lysis. 1 Publication
Mutagenesisi58 – 581L → E: No loss of function. 1 Publication
Mutagenesisi63 – 631K → E: No loss of function. 1 Publication
Mutagenesisi65 – 651W → E: Complete loss of function. Lysis. 1 Publication
Mutagenesisi66 – 661K → D: No loss of function. 1 Publication
Mutagenesisi66 – 661K → Q: Loss of glycation mediated inactivation. 1 Publication
Mutagenesisi67 – 671F → K: No loss of function. 1 Publication
Mutagenesisi69 – 691H → Q: Loss of glycation mediated inactivation. 1 Publication
Mutagenesisi72 – 721F → E: Almost complete loss of function. Lysis. 1 Publication
Mutagenesisi78 – 781R → E: Loss of function. Lysis. 1 Publication
Mutagenesisi79 – 791L → D: No loss of function. 1 Publication
Mutagenesisi81 – 811E → R: Almost complete loss of function. Lysis. 1 Publication
Mutagenesisi82 – 821N → K: No loss of function. 1 Publication
Mutagenesisi87 – 871Y → R: No loss of function. 1 Publication

Keywords - Diseasei

Disease mutation, Hereditary hemolytic anemia

Organism-specific databases

MalaCardsiCD59.
MIMi612300. phenotype.
Orphaneti169464. Primary CD59 deficiency.
PharmGKBiPA26228.

Polymorphism and mutation databases

BioMutaiCD59.
DMDMi116021.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 2525Combined sources1 PublicationAdd
BLAST
Chaini26 – 10277CD59 glycoproteinPRO_0000036108Add
BLAST
Propeptidei103 – 12826Removed in mature form1 PublicationPRO_0000036109Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Disulfide bondi28 ↔ 511 Publication
Disulfide bondi31 ↔ 381 Publication
Glycosylationi43 – 431N-linked (GlcNAc...)2 Publications
Disulfide bondi44 ↔ 641 Publication
Glycosylationi66 – 661N-linked (Glc) (glycation)
Disulfide bondi70 ↔ 881 Publication
Glycosylationi76 – 761O-linked (GalNAc...)Curated
Glycosylationi77 – 771O-linked (GalNAc...)Curated
Disulfide bondi89 ↔ 941 Publication
Lipidationi102 – 1021GPI-anchor amidated asparagine2 Publications

Post-translational modificationi

N- and O-glycosylated. The N-glycosylation mainly consists of a family of biantennary complex-type structures with and without lactosamine extensions and outer arm fucose residues. Also significant amounts of triantennary complexes (22%). Variable sialylation also present in the Asn-43 oligosaccharide. The predominant O-glycans are mono-sialylated forms of the disaccharide, Gal-beta-1,3GalNAc, and their sites of attachment are probably on Thr-76 and 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 and glucosamine. Sialic acid linked to an N-acetylhexosamine-galactose arm is present in two variants.2 Publications
Glycated. Glycation is found in diabetic subjects, but only at minimal levels in nondiabetic subjects. Glycated CD59 lacks MAC-inhibitory function and confers to vascular complications of diabetes.

Keywords - PTMi

Disulfide bond, Glycation, Glycoprotein, GPI-anchor, Lipoprotein

Proteomic databases

EPDiP13987.
PaxDbiP13987.
PeptideAtlasiP13987.
PRIDEiP13987.

PTM databases

iPTMnetiP13987.
PhosphoSiteiP13987.
SwissPalmiP13987.
UniCarbKBiP13987.

Expressioni

Gene expression databases

BgeeiENSG00000085063.
CleanExiHS_CD59.
ExpressionAtlasiP13987. baseline and differential.
GenevisibleiP13987. HS.

Organism-specific databases

HPAiCAB001448.
HPA026494.

Interactioni

Subunit structurei

Interacts with T-cell surface antigen CD2.1 Publication

Binary interactionsi

WithEntry#Exp.IntActNotes
CQ778I94EBI-297972,EBI-8716052From a different organism.
TMED10O355875EBI-297972,EBI-4405327From a different organism.
TMED2Q153634EBI-297972,EBI-998485

GO - Molecular functioni

Protein-protein interaction databases

BioGridi107404. 33 interactions.
IntActiP13987. 25 interactions.
MINTiMINT-5000502.
STRINGi9606.ENSP00000340210.

Structurei

Secondary structure

1
128
Legend: HelixTurnBeta strand
Show more details
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Beta strandi27 – 293Combined sources
Beta strandi32 – 343Combined sources
Beta strandi41 – 433Combined sources
Beta strandi50 – 567Combined sources
Beta strandi59 – 657Combined sources
Helixi67 – 693Combined sources
Helixi72 – 798Combined sources
Beta strandi85 – 895Combined sources
Beta strandi91 – 933Combined sources
Helixi97 – 993Combined sources

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
1CDQNMR-A26-102[»]
1CDRNMR-A26-102[»]
1CDSNMR-A26-102[»]
1ERGNMR-A26-95[»]
1ERHNMR-A26-95[»]
2J8BX-ray1.15A26-103[»]
2OFSX-ray2.12A26-99[»]
2UWRX-ray1.34A26-102[»]
2UX2X-ray1.80A/B/C26-102[»]
4BIKX-ray3.49B/D26-102[»]
ProteinModelPortaliP13987.
SMRiP13987. Positions 26-102.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP13987.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini26 – 10883UPAR/Ly6Add
BLAST

Sequence similaritiesi

Contains 1 UPAR/Ly6 domain.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiENOG410J39P. Eukaryota.
ENOG410ZEQP. LUCA.
GeneTreeiENSGT00390000016309.
HOGENOMiHOG000232180.
HOVERGENiHBG005284.
InParanoidiP13987.
KOiK04008.
OMAiNKAAFSI.
PhylomeDBiP13987.
TreeFamiTF338524.

Family and domain databases

InterProiIPR018363. CD59_antigen_CS.
IPR027101. CD59_glyco.
IPR016054. LY6_UPA_recep-like.
[Graphical view]
PANTHERiPTHR10036:SF9. PTHR10036:SF9. 1 hit.
PfamiPF00021. UPAR_LY6. 1 hit.
[Graphical view]
SMARTiSM00134. LU. 1 hit.
[Graphical view]
PROSITEiPS00983. LY6_UPAR. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P13987-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MGIQGGSVLF GLLLVLAVFC HSGHSLQCYN CPNPTADCKT AVNCSSDFDA
60 70 80 90 100
CLITKAGLQV YNKCWKFEHC NFNDVTTRLR ENELTYYCCK KDLCNFNEQL
110 120
ENGGTSLSEK TVLLLVTPFL AAAWSLHP
Length:128
Mass (Da):14,177
Last modified:January 1, 1990 - v1
Checksum:i2F0D29CBE3C28505
GO

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti89 – 891C → Y in HACD59. 1 Publication
Corresponds to variant rs397514767 [ dbSNP | Ensembl ].
VAR_070124

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M27909 mRNA. Translation: AAA60543.1.
M95708 mRNA. Translation: AAA60957.1.
X16447 mRNA. Translation: CAA34467.1.
X17198 mRNA. Translation: CAA35059.1.
M34671 mRNA. Translation: AAA51952.1.
M84345 Genomic DNA. No translation available.
M84349, M84346, M84348 Genomic DNA. Translation: AAA88793.1.
Z14113, Z14114, Z14115 Genomic DNA. Translation: CAA78486.1.
BT007104 mRNA. Translation: AAP35768.1.
BC001506 mRNA. Translation: AAH01506.1.
CCDSiCCDS7886.1.
PIRiA46252. RWHU59.
RefSeqiNP_000602.1. NM_000611.5.
NP_001120695.1. NM_001127223.1.
NP_001120697.1. NM_001127225.1.
NP_001120698.1. NM_001127226.1.
NP_001120699.1. NM_001127227.1.
NP_976074.1. NM_203329.2.
NP_976075.1. NM_203330.2.
NP_976076.1. NM_203331.2.
UniGeneiHs.278573.
Hs.709466.
Hs.710641.

Genome annotation databases

EnsembliENST00000351554; ENSP00000340210; ENSG00000085063.
ENST00000395850; ENSP00000379191; ENSG00000085063.
ENST00000415002; ENSP00000404822; ENSG00000085063.
ENST00000426650; ENSP00000402425; ENSG00000085063.
ENST00000437761; ENSP00000410182; ENSG00000085063.
ENST00000445143; ENSP00000403511; ENSG00000085063.
ENST00000525763; ENSP00000435179; ENSG00000085063.
ENST00000527577; ENSP00000432942; ENSG00000085063.
ENST00000528700; ENSP00000434617; ENSG00000085063.
GeneIDi966.
KEGGihsa:966.

Cross-referencesi

Web resourcesi

CD59base

CD59 mutation db

Atlas of Genetics and Cytogenetics in Oncology and Haematology

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M27909 mRNA. Translation: AAA60543.1.
M95708 mRNA. Translation: AAA60957.1.
X16447 mRNA. Translation: CAA34467.1.
X17198 mRNA. Translation: CAA35059.1.
M34671 mRNA. Translation: AAA51952.1.
M84345 Genomic DNA. No translation available.
M84349, M84346, M84348 Genomic DNA. Translation: AAA88793.1.
Z14113, Z14114, Z14115 Genomic DNA. Translation: CAA78486.1.
BT007104 mRNA. Translation: AAP35768.1.
BC001506 mRNA. Translation: AAH01506.1.
CCDSiCCDS7886.1.
PIRiA46252. RWHU59.
RefSeqiNP_000602.1. NM_000611.5.
NP_001120695.1. NM_001127223.1.
NP_001120697.1. NM_001127225.1.
NP_001120698.1. NM_001127226.1.
NP_001120699.1. NM_001127227.1.
NP_976074.1. NM_203329.2.
NP_976075.1. NM_203330.2.
NP_976076.1. NM_203331.2.
UniGeneiHs.278573.
Hs.709466.
Hs.710641.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
1CDQNMR-A26-102[»]
1CDRNMR-A26-102[»]
1CDSNMR-A26-102[»]
1ERGNMR-A26-95[»]
1ERHNMR-A26-95[»]
2J8BX-ray1.15A26-103[»]
2OFSX-ray2.12A26-99[»]
2UWRX-ray1.34A26-102[»]
2UX2X-ray1.80A/B/C26-102[»]
4BIKX-ray3.49B/D26-102[»]
ProteinModelPortaliP13987.
SMRiP13987. Positions 26-102.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107404. 33 interactions.
IntActiP13987. 25 interactions.
MINTiMINT-5000502.
STRINGi9606.ENSP00000340210.

PTM databases

iPTMnetiP13987.
PhosphoSiteiP13987.
SwissPalmiP13987.
UniCarbKBiP13987.

Polymorphism and mutation databases

BioMutaiCD59.
DMDMi116021.

Proteomic databases

EPDiP13987.
PaxDbiP13987.
PeptideAtlasiP13987.
PRIDEiP13987.

Protocols and materials databases

DNASUi966.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000351554; ENSP00000340210; ENSG00000085063.
ENST00000395850; ENSP00000379191; ENSG00000085063.
ENST00000415002; ENSP00000404822; ENSG00000085063.
ENST00000426650; ENSP00000402425; ENSG00000085063.
ENST00000437761; ENSP00000410182; ENSG00000085063.
ENST00000445143; ENSP00000403511; ENSG00000085063.
ENST00000525763; ENSP00000435179; ENSG00000085063.
ENST00000527577; ENSP00000432942; ENSG00000085063.
ENST00000528700; ENSP00000434617; ENSG00000085063.
GeneIDi966.
KEGGihsa:966.

Organism-specific databases

CTDi966.
GeneCardsiCD59.
H-InvDBHIX0171358.
HGNCiHGNC:1689. CD59.
HPAiCAB001448.
HPA026494.
MalaCardsiCD59.
MIMi107271. gene.
612300. phenotype.
neXtProtiNX_P13987.
Orphaneti169464. Primary CD59 deficiency.
PharmGKBiPA26228.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410J39P. Eukaryota.
ENOG410ZEQP. LUCA.
GeneTreeiENSGT00390000016309.
HOGENOMiHOG000232180.
HOVERGENiHBG005284.
InParanoidiP13987.
KOiK04008.
OMAiNKAAFSI.
PhylomeDBiP13987.
TreeFamiTF338524.

Enzyme and pathway databases

ReactomeiR-HSA-204005. COPII (Coat Protein 2) Mediated Vesicle Transport.
R-HSA-5694530. Cargo concentration in the ER.
R-HSA-6807878. COPI-mediated anterograde transport.
R-HSA-977606. Regulation of Complement cascade.

Miscellaneous databases

ChiTaRSiCD59. human.
EvolutionaryTraceiP13987.
GeneWikiiCD59.
GenomeRNAii966.
PROiP13987.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000085063.
CleanExiHS_CD59.
ExpressionAtlasiP13987. baseline and differential.
GenevisibleiP13987. HS.

Family and domain databases

InterProiIPR018363. CD59_antigen_CS.
IPR027101. CD59_glyco.
IPR016054. LY6_UPA_recep-like.
[Graphical view]
PANTHERiPTHR10036:SF9. PTHR10036:SF9. 1 hit.
PfamiPF00021. UPAR_LY6. 1 hit.
[Graphical view]
SMARTiSM00134. LU. 1 hit.
[Graphical view]
PROSITEiPS00983. LY6_UPAR. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiCD59_HUMAN
AccessioniPrimary (citable) accession number: P13987
Entry historyi
Integrated into UniProtKB/Swiss-Prot: January 1, 1990
Last sequence update: January 1, 1990
Last modified: September 7, 2016
This is version 189 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human cell differentiation molecules
    CD nomenclature of surface proteins of human leucocytes and list of entries
  2. Human chromosome 11
    Human chromosome 11: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.