Component
Human CoQ monooxygenase / COQ6
Human CoQ monooxygenase / COQ6. 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
Six COQ6 mutations in 13 individuals were linked to early nephrotic syndrome and sensorineural deafness.
Experimental context and source evidence
- availability_state
- machinery_impairment Imported condition classification; unverified.
- evidence_span
- {"source_cache": "artifacts/coq10-research/21540551.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "3f01d7d237328376c3eb1ed5a3c2c1ee7561d78fb3890101ab5e8eb678c64def", "start_char": 0, "end_char": 1142, "text_sha256": "3f01d7d237328376c3eb1ed5a3c2c1ee7561d78fb3890101ab5e8eb678c64def"}
- experimental_model
- Human pedigrees, yeast validation, podocyte and zebrafish knockdown
- exposure
- COQ6 variants; cell/embryo knockdown and CoQ treatment
- limitations
- Human disease association and model rescue are different evidence layers; not proof that every nephrotic syndrome responds to CoQ.
- nutrient_topic
- Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. · Coenzyme Q10 / CoQ10 redox system
- organism
- 13 humans from seven families plus model systems
- plain_language
- A renal presentation can arise from a CoQ synthesis gene.
- primary_references
- [coq10-p21540551] COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness. (2011). https://pubmed.ncbi.nlm.nih.gov/21540551/ DOI: 10.1172/jci45693
- tissue_or_cell_type
- Renal podocytes and inner-ear disease
- trigger_kind
- machinery_impairment Imported condition classification; unverified.
Coenzyme Q10: biosynthesis, electron transfer, antioxidant recycling and nutrient interactions (2026-09-17) · lines 1009–1020
AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Human pedigrees, yeast validation, podocyte and zebrafish knockdown · source_derived_draft · unverified_draft
### coq10-coq6-nephropathy Six COQ6 mutations in 13 individuals were linked to early nephrotic syndrome and sensorineural deafness. Condition category: machinery_impairment nutrient_topic: Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. plain_language: A renal presentation can arise from a CoQ synthesis gene. organism: 13 humans from seven families plus model systems tissue_or_cell_type: Renal podocytes and inner-ear disease experimental_model: Human pedigrees, yeast validation, podocyte and zebrafish knockdown limitations: Human disease association and model rescue are different evidence layers; not proof that every nephrotic syndrome responds to CoQ. exposure: COQ6 variants; cell/embryo knockdown and CoQ treatment evidence_span: {"source_cache": "artifacts/coq10-research/21540551.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "3f01d7d237328376c3eb1ed5a3c2c1ee7561d78fb3890101ab5e8eb678c64def", "start_char": 0, "end_char": 1142, "text_sha256": "3f01d7d237328376c3eb1ed5a3c2c1ee7561d78fb3890101ab5e8eb678c64def"} [coq10-p21540551] COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness. (2011). https://pubmed.ncbi.nlm.nih.gov/21540551/ DOI: 10.1172/jci45693
Complete structured claim and evidence
Where it participates (unsigned role)
Median protein-to-creatinine ratio fell from 1.66 to 0.19 g/gCr at 12 months; six of seven patients with 12-month data had at least a 50% reduction.
Experimental context and source evidence
- availability_state
- machinery_impairment Imported condition classification; unverified.
- evidence_span
- {"source_cache": "artifacts/coq10-research/42435122.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "59862dedc0e8ce5d72cf95f5f04a1d16e447cc16e9f2310fac81d15fb1fa9aa4", "start_char": 0, "end_char": 1715, "text_sha256": "59862dedc0e8ce5d72cf95f5f04a1d16e447cc16e9f2310fac81d15fb1fa9aa4"}
- experimental_model
- Retrospective longitudinal clinical cohort
- exposure
- CoQ supplementation; median initial dose 10 mg/kg/day
- limitations
- 2026 observational cohort without untreated randomization; incomplete 12-month data and genotype-specific context.
- nutrient_topic
- Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. · Coenzyme Q10 / CoQ10 redox system
- organism
- 12 Japanese patients: 11 COQ8B and one COQ6
- plain_language
- The treatment signal was in a genetically defined disease, not routine tiredness.
- primary_references
- [coq10-p42435122] Kidney outcomes of coenzyme Q10 supplementation in patients with genetically confirmed CoQ10 nephropathy in Japan. (2026). https://pubmed.ncbi.nlm.nih.gov/42435122/ DOI: 10.1007/s10157-026-02917-7
- tissue_or_cell_type
- Genetically confirmed CoQ nephropathy
- trigger_kind
- machinery_impairment Imported condition classification; unverified.
Coenzyme Q10: biosynthesis, electron transfer, antioxidant recycling and nutrient interactions (2026-09-17) · lines 1048–1059
AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Retrospective longitudinal clinical cohort · source_derived_draft · unverified_draft
### coq10-nephropathy-proteinuria Median protein-to-creatinine ratio fell from 1.66 to 0.19 g/gCr at 12 months; six of seven patients with 12-month data had at least a 50% reduction. Condition category: machinery_impairment nutrient_topic: Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. plain_language: The treatment signal was in a genetically defined disease, not routine tiredness. organism: 12 Japanese patients: 11 COQ8B and one COQ6 tissue_or_cell_type: Genetically confirmed CoQ nephropathy experimental_model: Retrospective longitudinal clinical cohort limitations: 2026 observational cohort without untreated randomization; incomplete 12-month data and genotype-specific context. exposure: CoQ supplementation; median initial dose 10 mg/kg/day evidence_span: {"source_cache": "artifacts/coq10-research/42435122.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "59862dedc0e8ce5d72cf95f5f04a1d16e447cc16e9f2310fac81d15fb1fa9aa4", "start_char": 0, "end_char": 1715, "text_sha256": "59862dedc0e8ce5d72cf95f5f04a1d16e447cc16e9f2310fac81d15fb1fa9aa4"} [coq10-p42435122] Kidney outcomes of coenzyme Q10 supplementation in patients with genetically confirmed CoQ10 nephropathy in Japan. (2026). https://pubmed.ncbi.nlm.nih.gov/42435122/ DOI: 10.1007/s10157-026-02917-7
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.