Entity Details

Primary name SUCB1_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionQ9P2R7
EntryNameSUCB1_HUMAN
FullNameSuccinate--CoA ligase [ADP-forming] subunit beta, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length463
SequenceStatuscomplete
DateCreated2002-05-27
DateModified2021-06-02

Ontological Relatives

GenesSUCLA2

GO terms

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GOName
GO:0000287 magnesium ion binding
GO:0004775 succinate-CoA ligase (ADP-forming) activity
GO:0004776 succinate-CoA ligase (GDP-forming) activity
GO:0005524 ATP binding
GO:0005739 mitochondrion
GO:0005759 mitochondrial matrix
GO:0006099 tricarboxylic acid cycle
GO:0006104 succinyl-CoA metabolic process
GO:0006781 succinyl-CoA pathway
GO:0042709 succinate-CoA ligase complex
GO:0070062 extracellular exosome

Subcellular Location

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Subcellular Location
Mitochondrion

Domains

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DomainNameCategoryType
IPR005809 Succinate--CoA synthetase, beta subunitFamilyFamily
IPR005811 ATP-citrate lyase/succinyl-CoA ligaseDomainDomain
IPR011761 ATP-grasp foldDomainDomain
IPR013650 ATP-grasp fold, succinyl-CoA synthetase-typeDomainDomain
IPR013815 ATP-grasp fold, subdomain 1FamilyHomologous superfamily
IPR016102 Succinyl-CoA synthetase-likeFamilyHomologous superfamily
IPR017866 Succinyl-CoA synthetase, beta subunit, conserved siteSiteConserved site
IPR034723 Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrialFamilyFamily

Diseases

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Disease IDSourceNameDescription
612073 OMIMMitochondrial DNA depletion syndrome 5 (MTDPS5)A disorder due to mitochondrial dysfunction. It is characterized by infantile onset of hypotonia, neurologic deterioration, a hyperkinetic-dystonic movement disorder, external ophthalmoplegia, deafness, variable renal tubular dysfunction, and mild methylmalonic aciduria in some patients. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB00787 AcyclovirDrugbanksmall molecule