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Protein

Beta-2-glycoprotein 1

Gene

APOH

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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

  • heparin binding Source: UniProtKB-KW
  • identical protein binding Source: IntAct
  • 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

Keywordsi

Molecular functionHeparin-binding

Enzyme and pathway databases

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

EuPathDBiHostDB:ENSG00000091583.10
HGNCiHGNC:616 APOH
MIMi138700 gene
neXtProtiNX_P02749

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Secreted

Pathology & Biotechi

Organism-specific databases

DisGeNETi350
OpenTargetsiENSG00000091583
PharmGKBiPA24903

Chemistry databases

DrugBankiDB05446 LJP 1082

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...) threonineBy similarity1
Disulfide bondi51 ↔ 79
Disulfide bondi84 ↔ 124
Disulfide bondi110 ↔ 137
Disulfide bondi142 ↔ 188
Glycosylationi149O-linked (GalNAc...) threonine1
Glycosylationi162N-linked (GlcNAc...) (complex) asparagine8 Publications1
Disulfide bondi174 ↔ 200
Glycosylationi183N-linked (GlcNAc...) asparagine4 Publications1
Glycosylationi193N-linked (GlcNAc...) asparagine4 Publications1
Disulfide bondi205 ↔ 248
Disulfide bondi234 ↔ 260
Glycosylationi253N-linked (GlcNAc...) asparagine5 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

EPDiP02749
MaxQBiP02749
PaxDbiP02749
PeptideAtlasiP02749
PRIDEiP02749
ProteomicsDBi51565
TopDownProteomicsiP02749

PTM databases

GlyConnecti678
iPTMnetiP02749
PhosphoSitePlusiP02749

Miscellaneous databases

PMAP-CutDBiP02749

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

Show more details

GO - Molecular functioni

  • identical protein binding Source: IntAct

Protein-protein interaction databases

BioGridi106847, 13 interactors
DIPiDIP-46878N
IntActiP02749, 15 interactors
MINTiP02749
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

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

Keywords - Domaini

Repeat, Signal, Sushi

Phylogenomic databases

eggNOGiKOG4297 Eukaryota
ENOG410XPJ1 LUCA
GeneTreeiENSGT00910000143999
HOGENOMiHOG000034008
HOVERGENiHBG004271
InParanoidiP02749
KOiK17305
OMAiKNKEKKC
OrthoDBiEOG091G0AFE
PhylomeDBiP02749
TreeFamiTF334137

Family and domain databases

CDDicd00033 CCP, 4 hits
InterProiView protein in InterPro
IPR035976 Sushi/SCR/CCP_sf
IPR015104 Sushi_2
IPR000436 Sushi_SCR_CCP_dom
PfamiView protein in Pfam
PF00084 Sushi, 4 hits
PF09014 Sushi_2, 1 hit
ProDomiView protein in ProDom or Entries sharing at least one domain
PD012422 Sushi_2, 1 hit
SMARTiView protein in SMART
SM00032 CCP, 4 hits
SUPFAMiSSF57535 SSF57535, 5 hits
PROSITEiView protein in PROSITE
PS50923 SUSHI, 4 hits

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 dbSNP:rs3826358Ensembl.1
Natural variantiVAR_008169107S → N in allele APOH*1. 3 PublicationsCorresponds to variant dbSNP:rs1801692Ensembl.1
Natural variantiVAR_019155154R → H1 PublicationCorresponds to variant dbSNP:rs8178847Ensembl.1
Natural variantiVAR_000673266V → L in 23% of the population. 2 PublicationsCorresponds to variant dbSNP:rs4581EnsemblClinVar.1
Natural variantiVAR_008170325C → G Loss of phosphatidylserine-binding. 1 PublicationCorresponds to variant dbSNP:rs1801689Ensembl.1
Natural variantiVAR_008171335W → S in allele APOH*3W; loss of phosphatidylserine-binding. 2 PublicationsCorresponds to variant dbSNP:rs1801690Ensembl.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

Similar proteinsi

Entry informationi

Entry nameiAPOH_HUMAN
AccessioniPrimary (citable) accession number: P02749
Secondary accession number(s): B2R9M3, Q9UCN7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: June 1, 1994
Last modified: July 18, 2018
This is version 207 of the entry and version 3 of the sequence. See 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

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