Component
Human decaprenyl-diphosphate synthase subunit 2 / PDSS2
Human decaprenyl-diphosphate synthase subunit 2 / PDSS2. Species, exposure and limitations are retained in each linked claim.
2 recorded relationships. Experimental role, claim status and evidence remain attached to each record.
How nutrients influence it
Every nutrient with a recorded effect on this component, credited to the nutrient that acted rather than the chapter that recorded it. Open a nutrient to see the findings and the conditions they were measured under.
Other things that act on it
Enzymes, hormones, genes, and other components with a recorded effect. These are not nutrients, so they do not count toward the arrows above. Each finding names the chapter that recorded it.
How nutrients reach it in more than one step
Chains of two or more recorded steps that end here, grouped by the nutrient they start from. Each step is a separate finding, so a chain is a route a mechanism could take, not proof that it does.
Tracing routes…
What it does
Every recorded relationship this component is part of, grouped by its role. Plain wording comes first; the technical statement follows.
What it acts on
Patient fibroblasts with PDSS2 variants had severely impaired decaprenyl-diphosphate synthesis.
Experimental context and source evidence
- availability_state
- machinery_impairment Imported condition classification; unverified.
- evidence_span
- {"source_cache": "artifacts/coq10-research/17186472.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "e9c50c316029827591ba4c9e6cc96b6a1b7b890f5b8dadfd88ac686c73d83865", "start_char": 0, "end_char": 1039, "text_sha256": "e9c50c316029827591ba4c9e6cc96b6a1b7b890f5b8dadfd88ac686c73d83865"}
- experimental_model
- Human genetics and radiolabeled-substrate assays
- exposure
- Compound heterozygous PDSS2 variants
- limitations
- Rare genetic disease; dietary shortage and tissue-wide thresholds were not established.
- nutrient_topic
- Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. · Coenzyme Q10 / CoQ10 redox system
- organism
- Human infant and patient fibroblasts
- plain_language
- The cell cannot finish CoQ normally if its tail-building machinery is impaired.
- primary_references
- [coq10-p17186472] Leigh syndrome with nephropathy and CoQ10 deficiency due to decaprenyl diphosphate synthase subunit 2 (PDSS2) mutations. (2006). https://pubmed.ncbi.nlm.nih.gov/17186472/ DOI: 10.1086/510023
- tissue_or_cell_type
- Muscle and fibroblast CoQ production
- trigger_kind
- machinery_impairment Imported condition classification; unverified.
Coenzyme Q10: biosynthesis, electron transfer, antioxidant recycling and nutrient interactions (2026-09-17) · lines 190–201
AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Human genetics and radiolabeled-substrate assays · source_derived_draft · unverified_draft
### coq10-pdss2 Patient fibroblasts with PDSS2 variants had severely impaired decaprenyl-diphosphate synthesis. Condition category: machinery_impairment nutrient_topic: Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. plain_language: The cell cannot finish CoQ normally if its tail-building machinery is impaired. organism: Human infant and patient fibroblasts tissue_or_cell_type: Muscle and fibroblast CoQ production experimental_model: Human genetics and radiolabeled-substrate assays limitations: Rare genetic disease; dietary shortage and tissue-wide thresholds were not established. exposure: Compound heterozygous PDSS2 variants evidence_span: {"source_cache": "artifacts/coq10-research/17186472.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "e9c50c316029827591ba4c9e6cc96b6a1b7b890f5b8dadfd88ac686c73d83865", "start_char": 0, "end_char": 1039, "text_sha256": "e9c50c316029827591ba4c9e6cc96b6a1b7b890f5b8dadfd88ac686c73d83865"} [coq10-p17186472] Leigh syndrome with nephropathy and CoQ10 deficiency due to decaprenyl diphosphate synthase subunit 2 (PDSS2) mutations. (2006). https://pubmed.ncbi.nlm.nih.gov/17186472/ DOI: 10.1086/510023
Complete structured claim and evidence
Where it participates (unsigned role)
The PDSS1 D308E variant was associated with deficient CoQ-dependent respiratory activity and defective functional complementation.
Experimental context and source evidence
- availability_state
- machinery_impairment Imported condition classification; unverified.
- evidence_span
- {"source_cache": "artifacts/coq10-research/17332895.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "0a500ba6d7c7b5f32817074367d71993504fbd9c5a10cb76038faf718fb79b87", "start_char": 0, "end_char": 1416, "text_sha256": "0a500ba6d7c7b5f32817074367d71993504fbd9c5a10cb76038faf718fb79b87"}
- experimental_model
- Pedigrees, respiratory assays and yeast complementation
- exposure
- PDSS1 D308E or COQ2 frameshift variants
- limitations
- Distinct families and mutations; quinone rescue in an assay is not equivalent to proven oral treatment of every organ.
- nutrient_topic
- Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. · Coenzyme Q10 / CoQ10 redox system
- organism
- Human families and yeast validation
- plain_language
- A second independent gene controls the same precursor supply route.
- primary_references
- [coq10-p17332895] Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders. (2007). https://pubmed.ncbi.nlm.nih.gov/17332895/ DOI: 10.1172/jci29089
- tissue_or_cell_type
- CoQ-dependent respiratory function
- trigger_kind
- machinery_impairment Imported condition classification; unverified.
Coenzyme Q10: biosynthesis, electron transfer, antioxidant recycling and nutrient interactions (2026-09-17) · lines 203–214
AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Pedigrees, respiratory assays and yeast complementation · source_derived_draft · unverified_draft
### coq10-pdss1 The PDSS1 D308E variant was associated with deficient CoQ-dependent respiratory activity and defective functional complementation. Condition category: machinery_impairment nutrient_topic: Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. plain_language: A second independent gene controls the same precursor supply route. organism: Human families and yeast validation tissue_or_cell_type: CoQ-dependent respiratory function experimental_model: Pedigrees, respiratory assays and yeast complementation limitations: Distinct families and mutations; quinone rescue in an assay is not equivalent to proven oral treatment of every organ. exposure: PDSS1 D308E or COQ2 frameshift variants evidence_span: {"source_cache": "artifacts/coq10-research/17332895.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "0a500ba6d7c7b5f32817074367d71993504fbd9c5a10cb76038faf718fb79b87", "start_char": 0, "end_char": 1416, "text_sha256": "0a500ba6d7c7b5f32817074367d71993504fbd9c5a10cb76038faf718fb79b87"} [coq10-p17332895] Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders. (2007). https://pubmed.ncbi.nlm.nih.gov/17332895/ DOI: 10.1172/jci29089
Complete structured claim and evidence
The events it takes part in
A mechanism often involves more than two components. These are the full events, with every participant and its role.
Situations it appears in
Low-supply and faulty-machinery situations recorded in the chapters where this component plays a part.
In the sources
Preserved passages that mention this component, quoted exactly. Open one to read it in context.
Open hypotheses
Proposed ideas that involve this component. They are labeled as hypotheses and do not change any recorded statement.
This is a research prototype built from draft material. It is not medical advice, and its statements still await verification against the original studies.