Component

Coenzyme Q biosynthesis

Coenzyme Q biosynthesis. Species, exposure and limitations are retained in each linked claim.

4 recorded relationships. Experimental role, claim status and evidence remain attached to each record.

Read recorded mechanisms Open recursive mind map

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.

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.

Recorded relationships

What acts on it

  1. A homozygous COQ2 variant caused severe impairment of CoQ10 synthesis in patient fibroblasts.

    Human 4-hydroxybenzoate polyprenyltransferase / COQ2 → Coenzyme Q biosynthesis source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    machinery_impairment Imported condition classification; unverified.
    evidence_span
    {"source_cache": "artifacts/coq10-research/16400613.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "bd16a213ac563cf32b275cdee32ba3082e6c296cf9129e0930e2f96f0d2dc72a", "start_char": 0, "end_char": 1000, "text_sha256": "bd16a213ac563cf32b275cdee32ba3082e6c296cf9129e0930e2f96f0d2dc72a"}
    experimental_model
    Family sequencing and fibroblast tracer assays
    exposure
    Homozygous COQ2 missense variant
    limitations
    Rare primary deficiency; sequence numbering follows the original publication.
    nutrient_topic
    Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. · Coenzyme Q10 / CoQ10 redox system
    organism
    Human siblings
    plain_language
    Having the building blocks does not help if the enzyme that joins them is defective.
    primary_references
    [coq10-p16400613] A mutation in para-hydroxybenzoate-polyprenyl transferase (COQ2) causes primary coenzyme Q10 deficiency. (2006). https://pubmed.ncbi.nlm.nih.gov/16400613/ DOI: 10.1086/500092
    tissue_or_cell_type
    CoQ synthesis
    trigger_kind
    machinery_impairment Imported condition classification; unverified.

    Coenzyme Q10: biosynthesis, electron transfer, antioxidant recycling and nutrient interactions (2026-09-17) · lines 229–240

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Family sequencing and fibroblast tracer assays · source_derived_draft · unverified_draft

    ### coq10-coq2-loss A homozygous COQ2 variant caused severe impairment of CoQ10 synthesis in patient fibroblasts. Condition category: machinery_impairment nutrient_topic: Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. plain_language: Having the building blocks does not help if the enzyme that joins them is defective. organism: Human siblings tissue_or_cell_type: CoQ synthesis experimental_model: Family sequencing and fibroblast tracer assays limitations: Rare primary deficiency; sequence numbering follows the original publication. exposure: Homozygous COQ2 missense variant evidence_span: {"source_cache": "artifacts/coq10-research/16400613.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "bd16a213ac563cf32b275cdee32ba3082e6c296cf9129e0930e2f96f0d2dc72a", "start_char": 0, "end_char": 1000, "text_sha256": "bd16a213ac563cf32b275cdee32ba3082e6c296cf9129e0930e2f96f0d2dc72a"} [coq10-p16400613] A mutation in para-hydroxybenzoate-polyprenyl transferase (COQ2) causes primary coenzyme Q10 deficiency. (2006). https://pubmed.ncbi.nlm.nih.gov/16400613/ DOI: 10.1086/500092
    Complete structured claim and evidence

  2. Human COQ3 expression rescued respiratory growth and partially restored CoQ synthesis in coq3-null yeast.

    Human CoQ O-methyltransferase / COQ3 → Coenzyme Q biosynthesis source_derived_draftungraded
    Experimental context and source evidence
    evidence_span
    {"source_cache": "artifacts/coq10-research/10777520.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "0acc430af6b08f94dcbd0165e149ff76f9ba6fbfc47fcc934a0476182b4534bd", "start_char": 0, "end_char": 1345, "text_sha256": "0acc430af6b08f94dcbd0165e149ff76f9ba6fbfc47fcc934a0476182b4534bd"}
    experimental_model
    Human gene expression in yeast and methyltransferase assays
    exposure
    Farnesylated substrate analogues
    limitations
    Engineered complementation and analogue assays; not a human methyl-donor supplementation trial.
    nutrient_topic
    Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. · Coenzyme Q10 / CoQ10 redox system
    organism
    Human COQ3 in yeast and cell-free assays
    plain_language
    The human enzyme could replace part of the missing yeast machinery.
    primary_references
    [coq10-p10777520] Isolation and functional expression of human COQ3, a gene encoding a methyltransferase required for ubiquinone biosynthesis. (2000). https://pubmed.ncbi.nlm.nih.gov/10777520/ DOI: 10.1074/jbc.275.17.12381
    tissue_or_cell_type
    Early and final CoQ intermediates

    Coenzyme Q10: biosynthesis, electron transfer, antioxidant recycling and nutrient interactions (2026-09-17) · lines 255–266

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Human gene expression in yeast and methyltransferase assays · source_derived_draft · unverified_draft

    ### coq10-coq3-rescue Human COQ3 expression rescued respiratory growth and partially restored CoQ synthesis in coq3-null yeast. Condition category: normal nutrient_topic: Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. plain_language: The human enzyme could replace part of the missing yeast machinery. organism: Human COQ3 in yeast and cell-free assays tissue_or_cell_type: Early and final CoQ intermediates experimental_model: Human gene expression in yeast and methyltransferase assays limitations: Engineered complementation and analogue assays; not a human methyl-donor supplementation trial. exposure: Farnesylated substrate analogues evidence_span: {"source_cache": "artifacts/coq10-research/10777520.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "0acc430af6b08f94dcbd0165e149ff76f9ba6fbfc47fcc934a0476182b4534bd", "start_char": 0, "end_char": 1345, "text_sha256": "0acc430af6b08f94dcbd0165e149ff76f9ba6fbfc47fcc934a0476182b4534bd"} [coq10-p10777520] Isolation and functional expression of human COQ3, a gene encoding a methyltransferase required for ubiquinone biosynthesis. (2000). https://pubmed.ncbi.nlm.nih.gov/10777520/ DOI: 10.1074/jbc.275.17.12381
    Complete structured claim and evidence

  3. The PDSS1 D308E variant was associated with deficient CoQ-dependent respiratory activity and defective functional complementation.

    Human decaprenyl-diphosphate synthase subunit 1 / PDSS1 → Coenzyme Q biosynthesis source_derived_draftungraded
    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

  4. Mitochondrial STARD7 preserved CoQ synthesis, oxidative phosphorylation and cristae organization.

    Human STARD7 lipid-transfer protein → Coenzyme Q biosynthesis source_derived_draftungraded
    Experimental context and source evidence
    evidence_span
    {"source_cache": "artifacts/coq10-research/36658222.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "8cd5c486ecb31ad03ea06a817eb5bc56e60abdfa8ae8d50a46502974be2898ce", "start_char": 0, "end_char": 1410, "text_sha256": "8cd5c486ecb31ad03ea06a817eb5bc56e60abdfa8ae8d50a46502974be2898ce"}
    experimental_model
    Protein processing, localization, transport and cell-growth experiments
    exposure
    PARL processing and compartment-specific STARD7 expression
    limitations
    Intracellular distribution mechanism; increasing oral dose does not prove delivery to a particular organelle.
    nutrient_topic
    Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. · Coenzyme Q10 / CoQ10 redox system
    organism
    Mammalian cell models
    plain_language
    The same protein has a different job when retained inside mitochondria.
    primary_references
    [coq10-p36658222] Mitochondria regulate intracellular coenzyme Q transport and ferroptotic resistance via STARD7. (2023). https://pubmed.ncbi.nlm.nih.gov/36658222/ DOI: 10.1038/s41556-022-01071-y
    tissue_or_cell_type
    Mitochondria and plasma membrane

    Coenzyme Q10: biosynthesis, electron transfer, antioxidant recycling and nutrient interactions (2026-09-17) · lines 489–500

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Protein processing, localization, transport and cell-growth experiments · source_derived_draft · unverified_draft

    ### coq10-stard7-mito Mitochondrial STARD7 preserved CoQ synthesis, oxidative phosphorylation and cristae organization. Condition category: normal nutrient_topic: Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect. plain_language: The same protein has a different job when retained inside mitochondria. organism: Mammalian cell models tissue_or_cell_type: Mitochondria and plasma membrane experimental_model: Protein processing, localization, transport and cell-growth experiments limitations: Intracellular distribution mechanism; increasing oral dose does not prove delivery to a particular organelle. exposure: PARL processing and compartment-specific STARD7 expression evidence_span: {"source_cache": "artifacts/coq10-research/36658222.abstract.txt", "locator": "Primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "8cd5c486ecb31ad03ea06a817eb5bc56e60abdfa8ae8d50a46502974be2898ce", "start_char": 0, "end_char": 1410, "text_sha256": "8cd5c486ecb31ad03ea06a817eb5bc56e60abdfa8ae8d50a46502974be2898ce"} [coq10-p36658222] Mitochondria regulate intracellular coenzyme Q transport and ferroptotic resistance via STARD7. (2023). https://pubmed.ncbi.nlm.nih.gov/36658222/ DOI: 10.1038/s41556-022-01071-y
    Complete structured claim and evidence

In the sources

Preserved passages that mention this component, quoted exactly. Open one to read it in context.

    This is a research prototype built from draft material. It is not medical advice, and its statements still await verification against the original studies.

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