Entity Details
Primary name |
DLP1_HUMAN |
Entity type |
UniProt |
Source |
Source Link |
Details
Accession | Q86YH6 |
EntryName | DLP1_HUMAN |
FullName | All trans-polyprenyl-diphosphate synthase PDSS2 |
TaxID | 9606 |
Evidence | evidence at protein level |
Length | 399 |
SequenceStatus | complete |
DateCreated | 2005-08-16 |
DateModified | 2021-06-02 |
Subcellular Location
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Subcellular Location |
Mitochondrion |
Domains
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Domain | Name | Category | Type |
IPR000092 | Polyprenyl synthetase | Family | Family |
IPR008949 | Isoprenoid synthase domain superfamily | Family | Homologous superfamily |
Diseases
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Disease ID | Source | Name | Description |
614652 | OMIM | Coenzyme 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