Entity Details

Primary name UCRI_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP47985
EntryNameUCRI_HUMAN
FullNameCytochrome b-c1 complex subunit Rieske, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length274
SequenceStatuscomplete
DateCreated1996-02-01
DateModified2021-06-02

Ontological Relatives

GenesUQCRFS1

GO terms

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GOName
GO:0005739 mitochondrion
GO:0005743 mitochondrial inner membrane
GO:0005750 mitochondrial respiratory chain complex III
GO:0005751 mitochondrial respiratory chain complex IV
GO:0006122 mitochondrial electron transport, ubiquinol to cytochrome c
GO:0008121 ubiquinol-cytochrome-c reductase activity
GO:0016021 integral component of membrane
GO:0016491 oxidoreductase activity
GO:0022904 respiratory electron transport chain
GO:0034551 mitochondrial respiratory chain complex III assembly
GO:0046872 metal ion binding
GO:0051537 2 iron, 2 sulfur cluster binding

Subcellular Location

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Subcellular Location
Mitochondrion inner membrane

Domains

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DomainNameCategoryType
IPR004192 Cytochrome b-c1 complex subunit Rieske, transmembrane domainDomainDomain
IPR005805 Rieske iron-sulphur protein, C-terminalDomainDomain
IPR006317 Ubiquinol-cytochrome c reductase, iron-sulphur subunitDomainDomain
IPR011070 Globular protein, non-globular alpha/beta subunitFamilyHomologous superfamily
IPR014349 Rieske iron-sulphur proteinFamilyFamily
IPR015248 Ubiquinol-cytochrome c reductase 8kDa, N-terminalDomainDomain
IPR017941 Rieske [2Fe-2S] iron-sulphur domainDomainDomain
IPR036922 Rieske [2Fe-2S] iron-sulphur domain superfamilyFamilyHomologous superfamily
IPR037008 Cytochrome bc1 complex subunit Rieske, transmembrane domain superfamilyFamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
618775 OMIMMitochondrial complex III deficiency, nuclear 10 (MC3DN10)A form of mitochondrial complex III deficiency, a disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. MC3DN10 is an autosomal recessive form characterized by fetal bradycardia, poor feeding, hypotonia, hypertrophic cardiomyopathy, alopecia totalis, low mitochondrial complex III activity and lactic acidosis. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB04141 2-Hexyloxy-6-Hydroxymethyl-Tetrahydro-Pyran-3,4,5-TriolDrugbanksmall molecule
DB04799 6-Hydroxy-5-undecyl-4,7-benzothiazoledioneDrugbanksmall molecule
DB07401 AzoxystrobinDrugbanksmall molecule
DB07636 5-Heptyl-6-hydroxy-1,3-benzothiazole-4,7-dioneDrugbanksmall molecule
DB07763 (5S)-3-ANILINO-5-(2,4-DIFLUOROPHENYL)-5-METHYL-1,3-OXAZOLIDINE-2,4-DIONEDrugbanksmall molecule
DB07778 (S)-famoxadoneDrugbanksmall molecule
DB08330 METHYL (2Z)-3-METHOXY-2-{2-[(E)-2-PHENYLVINYL]PHENYL}ACRYLATEDrugbanksmall molecule
DB08453 2-Nonyl-4-quinolinol 1-oxideDrugbanksmall molecule
DB08690 Ubiquinone Q2Drugbanksmall molecule