InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: PDSS1

Summary

InnateDB Protein

IDBP-63904.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

PDSS1

Protein Name

prenyl (decaprenyl) diphosphate synthase, subunit 1

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000365388

InnateDB Gene

IDBG-63902 (PDSS1)

Protein Structure

UniProt Annotation

Function

Supplies decaprenyl diphosphate, the precursor for the side chain of the isoprenoid quinones ubiquinone-10. {ECO:0000269PubMed:16262699}.

Subcellular Localization

Mitochondrion {ECO:0000305}.

Disease Associations

Coenzyme Q10 deficiency, primary, 2 (COQ10D2) [MIM:614651]: An autosomal recessive multisystem disorder characterized by early-onset deafness, optic atrophy, mild mental retardation, peripheral neuropathy, obesity, livedo reticularis, and cardiac valvulopathy. {ECO:0000269PubMed:17332895}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 3 experimentally validated interaction(s) in this database.

Experimentally validated
Total	3 [view]
Protein-Protein	1 [view]
Protein-DNA	2 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0000010	trans-hexaprenyltranstransferase activity
GO:0046872	metal ion binding
GO:0046982	protein heterodimerization activity
GO:0050347	trans-octaprenyltranstransferase activity

Biological Process

GO:0006744	ubiquinone biosynthetic process
GO:0008299	isoprenoid biosynthetic process
GO:0044281	small molecule metabolic process
GO:0051290	protein heterotetramerization