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Protein

Beta-2-glycoprotein 1

Gene

APOH

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

Functioni

Binds to various kinds of negatively charged substances such as heparin, phospholipids, and dextran sulfate. May prevent activation of the intrinsic blood coagulation cascade by binding to phospholipids on the surface of damaged cells.

GO - Molecular functioni

  • glycoprotein binding Source: BHF-UCL
  • heparin binding Source: UniProtKB-KW
  • lipid binding Source: BHF-UCL
  • lipoprotein lipase activator activity Source: BHF-UCL
  • phospholipid binding Source: BHF-UCL

GO - Biological processi

  • blood coagulation, intrinsic pathway Source: BHF-UCL
  • negative regulation of angiogenesis Source: BHF-UCL
  • negative regulation of blood coagulation Source: BHF-UCL
  • negative regulation of endothelial cell migration Source: BHF-UCL
  • negative regulation of endothelial cell proliferation Source: BHF-UCL
  • negative regulation of fibrinolysis Source: BHF-UCL
  • negative regulation of myeloid cell apoptotic process Source: BHF-UCL
  • negative regulation of smooth muscle cell apoptotic process Source: BHF-UCL
  • plasminogen activation Source: BHF-UCL
  • platelet degranulation Source: Reactome
  • positive regulation of blood coagulation Source: BHF-UCL
  • positive regulation of lipoprotein lipase activity Source: BHF-UCL
  • regulation of fibrinolysis Source: BHF-UCL
  • triglyceride metabolic process Source: BHF-UCL
  • triglyceride transport Source: BHF-UCL
Complete GO annotation...

Keywords - Ligandi

Heparin-binding

Enzyme and pathway databases

BioCyciZFISH:ENSG00000091583-MONOMER.
ReactomeiR-HSA-114608. Platelet degranulation.

Names & Taxonomyi

Protein namesi
Recommended name:
Beta-2-glycoprotein 1
Alternative name(s):
APC inhibitor
Activated protein C-binding protein
Anticardiolipin cofactor
Apolipoprotein H
Short name:
Apo-H
Beta-2-glycoprotein I
Short name:
B2GPI
Short name:
Beta(2)GPI
Gene namesi
Name:APOH
Synonyms:B2G1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 17

Organism-specific databases

HGNCiHGNC:616. APOH.

Subcellular locationi

GO - Cellular componenti

  • cell surface Source: BHF-UCL
  • chylomicron Source: BHF-UCL
  • extracellular exosome Source: UniProtKB
  • extracellular region Source: Reactome
  • extracellular space Source: UniProtKB
  • high-density lipoprotein particle Source: BHF-UCL
  • platelet dense granule lumen Source: Reactome
  • very-low-density lipoprotein particle Source: BHF-UCL
Complete GO annotation...

Keywords - Cellular componenti

Secreted

Pathology & Biotechi

Organism-specific databases

DisGeNETi350.
OpenTargetsiENSG00000091583.
PharmGKBiPA24903.

Polymorphism and mutation databases

BioMutaiAPOH.
DMDMi543826.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 194 PublicationsAdd BLAST19
ChainiPRO_000000205920 – 345Beta-2-glycoprotein 1Add BLAST326

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi23 ↔ 66
Glycosylationi33O-linked (GalNAc...)By similarity1
Disulfide bondi51 ↔ 79
Disulfide bondi84 ↔ 124
Disulfide bondi110 ↔ 137
Disulfide bondi142 ↔ 188
Glycosylationi149O-linked (GalNAc...)1
Glycosylationi162N-linked (GlcNAc...) (complex)8 Publications1
Disulfide bondi174 ↔ 200
Glycosylationi183N-linked (GlcNAc...)4 Publications1
Glycosylationi193N-linked (GlcNAc...)4 Publications1
Disulfide bondi205 ↔ 248
Disulfide bondi234 ↔ 260
Glycosylationi253N-linked (GlcNAc...)5 Publications1
Disulfide bondi264 ↔ 315
Disulfide bondi300 ↔ 325
Disulfide bondi307 ↔ 345

Post-translational modificationi

N- and O-glycosylated. PubMed:6587378 also reports glycosylation on 'Asn-188' for their allele.8 Publications

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

MaxQBiP02749.
PaxDbiP02749.
PeptideAtlasiP02749.
PRIDEiP02749.
TopDownProteomicsiP02749.

PTM databases

iPTMnetiP02749.
PhosphoSitePlusiP02749.

Miscellaneous databases

PMAP-CutDBP02749.

Expressioni

Tissue specificityi

Expressed by the liver and secreted in plasma.

Gene expression databases

BgeeiENSG00000091583.
CleanExiHS_APOH.
ExpressionAtlasiP02749. baseline and differential.
GenevisibleiP02749. HS.

Organism-specific databases

HPAiCAB022214.
HPA001654.
HPA003732.

Interactioni

Binary interactionsi

WithEntry#Exp.IntActNotes
itself2EBI-2114682,EBI-2114682
LPAP085194EBI-2114682,EBI-9232288
PF4P027762EBI-2114682,EBI-2565740
PLGP007472EBI-2114682,EBI-999394

Protein-protein interaction databases

BioGridi106847. 12 interactors.
DIPiDIP-46878N.
IntActiP02749. 12 interactors.
MINTiMINT-6743724.
STRINGi9606.ENSP00000205948.

Structurei

Secondary structure

1345
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi22 – 24Combined sources3
Beta strandi32 – 36Combined sources5
Beta strandi39 – 41Combined sources3
Beta strandi46 – 51Combined sources6
Beta strandi55 – 57Combined sources3
Beta strandi62 – 65Combined sources4
Beta strandi79 – 81Combined sources3
Beta strandi93 – 96Combined sources4
Beta strandi105 – 110Combined sources6
Beta strandi114 – 118Combined sources5
Beta strandi120 – 124Combined sources5
Beta strandi130 – 132Combined sources3
Beta strandi136 – 139Combined sources4
Beta strandi151 – 155Combined sources5
Beta strandi163 – 165Combined sources3
Beta strandi169 – 174Combined sources6
Beta strandi178 – 182Combined sources5
Beta strandi184 – 188Combined sources5
Beta strandi192 – 195Combined sources4
Beta strandi199 – 202Combined sources4
Beta strandi214 – 217Combined sources4
Beta strandi222 – 225Combined sources4
Beta strandi229 – 234Combined sources6
Beta strandi238 – 242Combined sources5
Beta strandi244 – 248Combined sources5
Beta strandi252 – 255Combined sources4
Beta strandi260 – 262Combined sources3
Beta strandi267 – 270Combined sources4
Beta strandi272 – 275Combined sources4
Beta strandi278 – 281Combined sources4
Helixi282 – 285Combined sources4
Turni286 – 288Combined sources3
Beta strandi295 – 302Combined sources8
Turni303 – 306Combined sources4
Beta strandi307 – 316Combined sources10
Turni331 – 333Combined sources3
Helixi339 – 341Combined sources3

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1C1ZX-ray2.87A20-345[»]
1G4FNMR-A261-345[»]
1G4GNMR-A261-345[»]
1QUBX-ray2.70A20-345[»]
2KRINMR-A263-345[»]
3OP8X-ray1.90A/B263-345[»]
4JHSX-ray3.00A203-345[»]
ProteinModelPortaliP02749.
SMRiP02749.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP02749.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini21 – 81Sushi 1PROSITE-ProRule annotationAdd BLAST61
Domaini82 – 139Sushi 2PROSITE-ProRule annotationAdd BLAST58
Domaini140 – 202Sushi 3PROSITE-ProRule annotationAdd BLAST63
Domaini203 – 262Sushi 4PROSITE-ProRule annotationAdd BLAST60

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni263 – 345Sushi-likeAdd BLAST83

Sequence similaritiesi

Contains 4 Sushi (CCP/SCR) domains.PROSITE-ProRule annotation

Keywords - Domaini

Repeat, Signal, Sushi

Phylogenomic databases

eggNOGiKOG4297. Eukaryota.
ENOG410XPJ1. LUCA.
GeneTreeiENSGT00850000132282.
HOGENOMiHOG000034008.
HOVERGENiHBG004271.
InParanoidiP02749.
KOiK17305.
OMAiRFTCPLT.
OrthoDBiEOG091G0AFE.
PhylomeDBiP02749.
TreeFamiTF334137.

Family and domain databases

CDDicd00033. CCP. 4 hits.
InterProiIPR015104. Sushi_2.
IPR000436. Sushi_SCR_CCP_dom.
[Graphical view]
PfamiPF00084. Sushi. 4 hits.
PF09014. Sushi_2. 1 hit.
[Graphical view]
ProDomiPD012422. Sushi_2. 1 hit.
[Graphical view] [Entries sharing at least one domain]
SMARTiSM00032. CCP. 4 hits.
[Graphical view]
SUPFAMiSSF57535. SSF57535. 5 hits.
PROSITEiPS50923. SUSHI. 4 hits.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

P02749-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MISPVLILFS SFLCHVAIAG RTCPKPDDLP FSTVVPLKTF YEPGEEITYS
60 70 80 90 100
CKPGYVSRGG MRKFICPLTG LWPINTLKCT PRVCPFAGIL ENGAVRYTTF
110 120 130 140 150
EYPNTISFSC NTGFYLNGAD SAKCTEEGKW SPELPVCAPI ICPPPSIPTF
160 170 180 190 200
ATLRVYKPSA GNNSLYRDTA VFECLPQHAM FGNDTITCTT HGNWTKLPEC
210 220 230 240 250
REVKCPFPSR PDNGFVNYPA KPTLYYKDKA TFGCHDGYSL DGPEEIECTK
260 270 280 290 300
LGNWSAMPSC KASCKVPVKK ATVVYQGERV KIQEKFKNGM LHGDKVSFFC
310 320 330 340
KNKEKKCSYT EDAQCIDGTI EVPKCFKEHS SLAFWKTDAS DVKPC
Length:345
Mass (Da):38,298
Last modified:June 1, 1994 - v3
Checksum:i63101704F8EDFE3F
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti121S → C AA sequence (PubMed:6587378).Curated1
Sequence conflicti188C → N AA sequence (PubMed:6587378).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0483165V → A.Corresponds to variant rs3826358dbSNPEnsembl.1
Natural variantiVAR_008169107S → N in allele APOH*1. 3 PublicationsCorresponds to variant rs1801692dbSNPEnsembl.1
Natural variantiVAR_019155154R → H.1 PublicationCorresponds to variant rs8178847dbSNPEnsembl.1
Natural variantiVAR_000673266V → L in 23% of the population. 2 PublicationsCorresponds to variant rs4581dbSNPEnsembl.1
Natural variantiVAR_008170325C → G Loss of phosphatidylserine-binding. 1 PublicationCorresponds to variant rs1801689dbSNPEnsembl.1
Natural variantiVAR_008171335W → S in allele APOH*3W; loss of phosphatidylserine-binding. 2 PublicationsCorresponds to variant rs1801690dbSNPEnsembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X58100 mRNA. Translation: CAA41113.1.
X53595 mRNA. Translation: CAA37664.1.
X57847 mRNA. Translation: CAA40977.1.
M62839 mRNA. Translation: AAA51766.1.
S80305 mRNA. Translation: AAB21330.1.
Y11493
, Y11494, Y11495, X53595, Y11496, Y11497, Y11498 Genomic DNA. Translation: CAA72279.1.
Y17754 Genomic DNA. Translation: CAA76845.1.
AK313838 mRNA. Translation: BAG36570.1.
AY322156 Genomic DNA. Translation: AAP72014.1.
BC020703 mRNA. Translation: AAH20703.1.
BC026283 mRNA. Translation: AAH26283.1.
CCDSiCCDS11663.1.
PIRiS17178. NBHU.
RefSeqiNP_000033.2. NM_000042.2.
UniGeneiHs.445358.

Genome annotation databases

EnsembliENST00000205948; ENSP00000205948; ENSG00000091583.
GeneIDi350.
KEGGihsa:350.
UCSCiuc002jfn.5. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Web resourcesi

SeattleSNPs
SHMPD

The Singapore human mutation and polymorphism database

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X58100 mRNA. Translation: CAA41113.1.
X53595 mRNA. Translation: CAA37664.1.
X57847 mRNA. Translation: CAA40977.1.
M62839 mRNA. Translation: AAA51766.1.
S80305 mRNA. Translation: AAB21330.1.
Y11493
, Y11494, Y11495, X53595, Y11496, Y11497, Y11498 Genomic DNA. Translation: CAA72279.1.
Y17754 Genomic DNA. Translation: CAA76845.1.
AK313838 mRNA. Translation: BAG36570.1.
AY322156 Genomic DNA. Translation: AAP72014.1.
BC020703 mRNA. Translation: AAH20703.1.
BC026283 mRNA. Translation: AAH26283.1.
CCDSiCCDS11663.1.
PIRiS17178. NBHU.
RefSeqiNP_000033.2. NM_000042.2.
UniGeneiHs.445358.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1C1ZX-ray2.87A20-345[»]
1G4FNMR-A261-345[»]
1G4GNMR-A261-345[»]
1QUBX-ray2.70A20-345[»]
2KRINMR-A263-345[»]
3OP8X-ray1.90A/B263-345[»]
4JHSX-ray3.00A203-345[»]
ProteinModelPortaliP02749.
SMRiP02749.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi106847. 12 interactors.
DIPiDIP-46878N.
IntActiP02749. 12 interactors.
MINTiMINT-6743724.
STRINGi9606.ENSP00000205948.

PTM databases

iPTMnetiP02749.
PhosphoSitePlusiP02749.

Polymorphism and mutation databases

BioMutaiAPOH.
DMDMi543826.

Proteomic databases

MaxQBiP02749.
PaxDbiP02749.
PeptideAtlasiP02749.
PRIDEiP02749.
TopDownProteomicsiP02749.

Protocols and materials databases

DNASUi350.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000205948; ENSP00000205948; ENSG00000091583.
GeneIDi350.
KEGGihsa:350.
UCSCiuc002jfn.5. human.

Organism-specific databases

CTDi350.
DisGeNETi350.
GeneCardsiAPOH.
HGNCiHGNC:616. APOH.
HPAiCAB022214.
HPA001654.
HPA003732.
MIMi138700. gene.
neXtProtiNX_P02749.
OpenTargetsiENSG00000091583.
PharmGKBiPA24903.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4297. Eukaryota.
ENOG410XPJ1. LUCA.
GeneTreeiENSGT00850000132282.
HOGENOMiHOG000034008.
HOVERGENiHBG004271.
InParanoidiP02749.
KOiK17305.
OMAiRFTCPLT.
OrthoDBiEOG091G0AFE.
PhylomeDBiP02749.
TreeFamiTF334137.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000091583-MONOMER.
ReactomeiR-HSA-114608. Platelet degranulation.

Miscellaneous databases

ChiTaRSiAPOH. human.
EvolutionaryTraceiP02749.
GeneWikiiApolipoprotein_H.
GenomeRNAii350.
PMAP-CutDBP02749.
PROiP02749.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000091583.
CleanExiHS_APOH.
ExpressionAtlasiP02749. baseline and differential.
GenevisibleiP02749. HS.

Family and domain databases

CDDicd00033. CCP. 4 hits.
InterProiIPR015104. Sushi_2.
IPR000436. Sushi_SCR_CCP_dom.
[Graphical view]
PfamiPF00084. Sushi. 4 hits.
PF09014. Sushi_2. 1 hit.
[Graphical view]
ProDomiPD012422. Sushi_2. 1 hit.
[Graphical view] [Entries sharing at least one domain]
SMARTiSM00032. CCP. 4 hits.
[Graphical view]
SUPFAMiSSF57535. SSF57535. 5 hits.
PROSITEiPS50923. SUSHI. 4 hits.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiAPOH_HUMAN
AccessioniPrimary (citable) accession number: P02749
Secondary accession number(s): B2R9M3, Q9UCN7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: June 1, 1994
Last modified: November 30, 2016
This is version 192 of the entry and version 3 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 chromosome 17
    Human chromosome 17: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. 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.