Entity Details

Primary name ODPB_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP11177
EntryNameODPB_HUMAN
FullNamePyruvate dehydrogenase E1 component subunit beta, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length359
SequenceStatuscomplete
DateCreated1989-07-01
DateModified2021-06-02

Ontological Relatives

GenesPDHB

GO terms

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GOName
GO:0004739 pyruvate dehydrogenase (acetyl-transferring) activity
GO:0005634 nucleus
GO:0005654 nucleoplasm
GO:0005739 mitochondrion
GO:0005759 mitochondrial matrix
GO:0005967 mitochondrial pyruvate dehydrogenase complex
GO:0006006 glucose metabolic process
GO:0006086 acetyl-CoA biosynthetic process from pyruvate
GO:0006090 pyruvate metabolic process
GO:0006099 tricarboxylic acid cycle
GO:0045254 pyruvate dehydrogenase complex
GO:0061732 mitochondrial acetyl-CoA biosynthetic process from pyruvate

Subcellular Location

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Subcellular Location
Mitochondrion matrix

Domains

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DomainNameCategoryType
IPR005475 Transketolase-like, pyrimidine-binding domainDomainDomain
IPR009014 Transketolase C-terminal/Pyruvate-ferredoxin oxidoreductase domain IIFamilyHomologous superfamily
IPR027110 Pyruvate dehydrogenase E1 component subunit betaFamilyFamily
IPR029061 Thiamin diphosphate-binding foldFamilyHomologous superfamily
IPR033248 Transketolase, C-terminal domainDomainDomain

Diseases

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Disease IDSourceNameDescription
614111 OMIMPyruvate dehydrogenase E1-beta deficiency (PDHBD)An enzymatic defect causing primary lactic acidosis in children. It is associated with a broad clinical spectrum ranging from fatal lactic acidosis in the newborn to chronic neurologic dysfunction with structural abnormalities in the central nervous system without systemic acidosis. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB00119 Pyruvic acidDrugbanksmall molecule
DB00157 NADHDrugbanksmall molecule

Interactions

28 interactions

InteractorPartnerSourcesPublicationsLink
ODPB_HUMANODPA_HUMANBioGRID, DIP, HPRD, IntAct12651851 19081061 22939629 26186194 26344197 28514442 29128334 31536960 34079125 7864652 details
ODPB_HUMANODP2_HUMANBioGRID, IntAct, MINT14638692 18206651 26186194 26344197 28514442 details
ODPB_HUMANFCERA_HUMANBioGRID, IntAct21988832 details
ODPB_HUMANGBB2_HUMANBioGRID, IntAct21988832 details
ODPB_HUMANITB1_HUMANBioGRID, IntAct21988832 details
ODPB_HUMANANXA7_HUMANBioGRID, MINT21900206 details
ODPB_HUMANCDN1A_HUMANBioGRID, MINT21900206 details
ODPB_HUMANGRB7_HUMANBioGRID, MINT21900206 details
ODPB_HUMANPFD1_HUMANBioGRID, MINT21900206 details
ODPB_HUMANRAP1B_HUMANBioGRID, MINT21900206 details
ODPB_HUMANRCC1_HUMANBioGRID, MINT21900206 details
ODPB_HUMANT22D1_HUMANBioGRID, MINT21900206 details
ODPB_HUMANAKT1_HUMANBioGRID, MINT25640309 details
ODPB_HUMANAA2AR_HUMANBioGRID, MINT28298427 details
ODPB_HUMANTRI63_HUMANBioGRID, HPRD15967462 18468620 details
ODPB_HUMANHCD2_HUMANBioGRID22496890 details
ODPB_HUMANBRF2_HUMANIntAct17353931 details
ODPB_HUMANWRP73_HUMANIntAct17353931 details
ODPB_HUMANCRCM_HUMANIntAct17353931 details
ODPB_HUMANSOMA_HUMANIntAct17353931 details
ODPB_HUMANIKKE_HUMANIntAct17353931 details
ODPB_HUMANCFA20_HUMANBioGRID, IntAct27173435 unassigned1312 details
ODPB_HUMANODPX_HUMANBioGRID, HPRD, IntAct22939629 26186194 28514442 7864652 details
ODPB_HUMANSHAN3_HUMANMINT21653829 details
ODPB_HUMANODPAT_HUMANBioGRID, HPRD26344197 7864652 details
ODPB_HUMANSRRM2_HUMANBioGRID16159877 details
ODPB_HUMANAKTIP_HUMANBioGRID27512140 details
ODPB_HUMANODPB_HUMANHPRD12651851 2377599 details