Entity Details

Primary name DLP1_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionQ86YH6
EntryNameDLP1_HUMAN
FullNameAll trans-polyprenyl-diphosphate synthase PDSS2
TaxID9606
Evidenceevidence at protein level
Length399
SequenceStatuscomplete
DateCreated2005-08-16
DateModified2021-06-02

Ontological Relatives

GenesPDSS2

GO terms

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GOName
GO:0000010 trans-hexaprenyltranstransferase activity
GO:0004659 prenyltransferase activity
GO:0005759 mitochondrial matrix
GO:0005829 cytosol
GO:0006744 ubiquinone biosynthetic process
GO:0008299 isoprenoid biosynthetic process
GO:0021549 cerebellum development
GO:0046982 protein heterodimerization activity
GO:0050347 trans-octaprenyltranstransferase activity
GO:0050878 regulation of body fluid levels
GO:0097269 all-trans-decaprenyl-diphosphate synthase activity
GO:1990234 transferase complex

Subcellular Location

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Subcellular Location
Mitochondrion

Domains

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DomainNameCategoryType
IPR000092 Polyprenyl synthetaseFamilyFamily
IPR008949 Isoprenoid synthase domain superfamilyFamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
614652 OMIMCoenzyme Q10 deficiency, primary, 3 (COQ10D3)A fatal encephalomyopathic form of coenzyme Q10 deficiency with nephrotic syndrome. Coenzyme Q10 deficiency is an autosomal recessive disorder with variable manifestations consistent with 5 major phenotypes. The phenotypes include an encephalomyopathic form with seizures and ataxia; a multisystem infantile form with encephalopathy, cardiomyopathy and renal failure; a predominantly cerebellar form with ataxia and cerebellar atrophy; Leigh syndrome with growth retardation; and an isolated myopathic form. The disease is caused by variants affecting the gene represented in this entry.

Interactions

1 interaction

InteractorPartnerSourcesPublicationsLink
DLP1_HUMANA4_HUMANBioGRID21832049 details