Pdss1 Gene Summary [Mouse]
Enables protein heterodimerization activity. Contributes to all-trans-nonaprenyl-diphosphate synthase (geranyl-diphosphate specific) activity. Involved in isoprenoid biosynthetic process and ubiquinone biosynthetic process. Located in mitochondrion. Part of heterotetrameric polyprenyl diphosphate synthase complex and ubiquinone biosynthesis complex. Is expressed in heart and heart right ventricle. Human ortholog(s) of this gene implicated in primary coenzyme Q10 deficiency 2. Orthologous to human PDSS1 (decaprenyl diphosphate synthase subunit 1). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Retained Intron |
| Official Symbol | Pdss1 |
| Official Name | prenyl (solanesyl) diphosphate synthase, subunit 1 [Source:MGI Symbol;Acc:MGI:1889278] |
| Ensembl ID | ENSMUSG00000026784 |
| Bio databases IDs | |
| Aliases | prenyl (solanesyl) diphosphate synthase, subunit 1 |
| Synonyms | 2610203G20Rik, 2700031G06Rik, COQ1, COQ10D2, COQ1A, decaprenyl diphosphate synthase subunit 1, DPS, hDPS1, mDLP1, mSPS1, prenyl (solanesyl) diphosphate synthase, subunit 1, SPS, TPRT, TPT, TPT 1, TPTF |
| Species | Mouse, Mus musculus |
| Orthologies |
Protein Domains
A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in mouse Pdss1 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- all-trans-decaprenyl-diphosphate synthase
- Isoprenoid Biosynthesis enzymes, Class 1
- prenyltransferase
- Polyprenyl synthetase
- enzyme
- protein binding
- protein heterodimerization
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- deafness-encephaloneuropathy-obesity-valvulopathy syndrome
- primary coenzyme Q10 deficiency type 2
- fatal infantile multiorgan disease
- idiopathic scoliosis
- CoQ10 deficiency
- overall survival
- productive infection by HIV-1
regulated by
- TP53
- TGF beta
- Salmonella enterica serotype abortus equi lipopolysaccharide
- mir-22 (includes others)
- arsenite
- benzo(a)pyrene
- STAR
- EAhy926 cells
regulates
- ubiquinone
- terpenoid
Subcellular Expression
Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
- Cytoplasm
- Mitochondria
- mitochondrial matrix
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Pdss1 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- isoprenoid biosynthetic process
- ubiquinone biosynthetic process
Cellular Component
Where in the cell the gene product is active
- mitochondrial matrix
- heterotetrameric decaprenyl diphosphate synthase complex
Molecular Function
What the gene product does at the molecular level
- protein binding
- all-trans-decaprenyl-diphosphate synthase activity
- prenyltransferase activity
- protein heterodimerization activity
- metal ion binding
- all-trans-nonaprenyl-diphosphate synthase (geranyl-diphosphate specific) activity