Component

3-Hydroxyisovalerylcarnitine / 3-HIA-carnitine

3-Hydroxyisovalerylcarnitine / 3-HIA-carnitine. Species, exposure and limitations are retained in each linked claim.

6 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

Where it participates (unsigned role)

  1. Biotin-deficient but carnitine-sufficient HepG2 cells had more than tenfold higher intracellular 3-hydroxyisovalerylcarnitine than doubly sufficient cells.

    Biotin → HepG2 3-hydroxyisovalerylcarnitine pool source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    nutrient_deficiency Imported condition classification; unverified.
    evidence_span
    {"source_cache": "artifacts/biotin-research/25527659.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "4a368eb2fa2020bb1cabc99b31a3420870a8256886d5934d3d834c90b078238f", "start_char": 0, "end_char": 2372, "text_sha256": "4a368eb2fa2020bb1cabc99b31a3420870a8256886d5934d3d834c90b078238f"}
    experimental_model
    Two-factor biotin/carnitine depletion and carnitine repletion in human HepG2 cells
    exposure
    Separate and combined biotin and carnitine depletion
    limitations
    This is a cell-culture demonstration of marker masking; it does not validate a diagnostic correction formula in pregnancy or the general population.
    nutrient_topic
    Biotin research collection; topical membership is not evidence of a direct dietary effect. · Biotin
    organism
    Homo sapiens
    plain_language
    When carnitine is available, the biotin-related bottleneck produces a strong side-product signal.
    primary_references
    [b7-p25527659] In HepG2 cells, coexisting carnitine deficiency masks important indicators of marginal biotin deficiency. (2015). https://pubmed.ncbi.nlm.nih.gov/25527659/ DOI: 10.3945/jn.114.201343
    tissue_or_cell_type
    HepG2 hepatoma cells
    trigger_kind
    nutrient_deficiency Imported condition classification; unverified.

    Biotin: carboxylases, recycling, deficiency and nutrient interactions (2026-09-17) · lines 949–960

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Two-factor biotin/carnitine depletion and carnitine repletion in human HepG2 cells · source_derived_draft · unverified_draft

    ### b7-c5oh-biotin-low Biotin-deficient but carnitine-sufficient HepG2 cells had more than tenfold higher intracellular 3-hydroxyisovalerylcarnitine than doubly sufficient cells. Condition category: nutrient_deficiency nutrient_topic: Biotin research collection; topical membership is not evidence of a direct dietary effect. plain_language: When carnitine is available, the biotin-related bottleneck produces a strong side-product signal. organism: Homo sapiens tissue_or_cell_type: HepG2 hepatoma cells experimental_model: Two-factor biotin/carnitine depletion and carnitine repletion in human HepG2 cells limitations: This is a cell-culture demonstration of marker masking; it does not validate a diagnostic correction formula in pregnancy or the general population. exposure: Separate and combined biotin and carnitine depletion evidence_span: {"source_cache": "artifacts/biotin-research/25527659.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "4a368eb2fa2020bb1cabc99b31a3420870a8256886d5934d3d834c90b078238f", "start_char": 0, "end_char": 2372, "text_sha256": "4a368eb2fa2020bb1cabc99b31a3420870a8256886d5934d3d834c90b078238f"} [b7-p25527659] In HepG2 cells, coexisting carnitine deficiency masks important indicators of marginal biotin deficiency. (2015). https://pubmed.ncbi.nlm.nih.gov/25527659/ DOI: 10.3945/jn.114.201343
    Complete structured claim and evidence

  2. Combined biotin and carnitine deficiency largely masked the increase in substrate-derived acylcarnitines observed with isolated biotin deficiency in HepG2 cells.

    L-Carnitine → HepG2 3-hydroxyisovalerylcarnitine pool source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    nutrient_deficiency Imported condition classification; unverified.
    evidence_span
    {"source_cache": "artifacts/biotin-research/25527659.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "4a368eb2fa2020bb1cabc99b31a3420870a8256886d5934d3d834c90b078238f", "start_char": 0, "end_char": 2372, "text_sha256": "4a368eb2fa2020bb1cabc99b31a3420870a8256886d5934d3d834c90b078238f"}
    experimental_model
    Two-factor biotin/carnitine depletion and carnitine repletion in human HepG2 cells
    exposure
    Separate and combined biotin and carnitine depletion
    limitations
    This is a cell-culture demonstration of marker masking; it does not validate a diagnostic correction formula in pregnancy or the general population.
    nutrient_topic
    Biotin research collection; topical membership is not evidence of a direct dietary effect. · Biotin
    organism
    Homo sapiens
    plain_language
    A second shortage can hide the laboratory signature of the first.
    primary_references
    [b7-p25527659] In HepG2 cells, coexisting carnitine deficiency masks important indicators of marginal biotin deficiency. (2015). https://pubmed.ncbi.nlm.nih.gov/25527659/ DOI: 10.3945/jn.114.201343
    tissue_or_cell_type
    HepG2 hepatoma cells
    trigger_kind
    nutrient_deficiency Imported condition classification; unverified.

    Biotin: carboxylases, recycling, deficiency and nutrient interactions (2026-09-17) · lines 962–973

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Two-factor biotin/carnitine depletion and carnitine repletion in human HepG2 cells · source_derived_draft · unverified_draft

    ### b7-carnitine-hides-c5oh Combined biotin and carnitine deficiency largely masked the increase in substrate-derived acylcarnitines observed with isolated biotin deficiency in HepG2 cells. Condition category: nutrient_deficiency nutrient_topic: Biotin research collection; topical membership is not evidence of a direct dietary effect. plain_language: A second shortage can hide the laboratory signature of the first. organism: Homo sapiens tissue_or_cell_type: HepG2 hepatoma cells experimental_model: Two-factor biotin/carnitine depletion and carnitine repletion in human HepG2 cells limitations: This is a cell-culture demonstration of marker masking; it does not validate a diagnostic correction formula in pregnancy or the general population. exposure: Separate and combined biotin and carnitine depletion evidence_span: {"source_cache": "artifacts/biotin-research/25527659.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "4a368eb2fa2020bb1cabc99b31a3420870a8256886d5934d3d834c90b078238f", "start_char": 0, "end_char": 2372, "text_sha256": "4a368eb2fa2020bb1cabc99b31a3420870a8256886d5934d3d834c90b078238f"} [b7-p25527659] In HepG2 cells, coexisting carnitine deficiency masks important indicators of marginal biotin deficiency. (2015). https://pubmed.ncbi.nlm.nih.gov/25527659/ DOI: 10.3945/jn.114.201343
    Complete structured claim and evidence

  3. Carnitine repletion of doubly deficient HepG2 cells increased acetylcarnitine, propionylcarnitine and 3-hydroxyisovalerylcarnitine each by more than 50-fold.

    L-Carnitine → HepG2 acylcarnitine response to carnitine repletion source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    nutrient_deficiency Imported condition classification; unverified.
    evidence_span
    {"source_cache": "artifacts/biotin-research/25527659.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "4a368eb2fa2020bb1cabc99b31a3420870a8256886d5934d3d834c90b078238f", "start_char": 0, "end_char": 2372, "text_sha256": "4a368eb2fa2020bb1cabc99b31a3420870a8256886d5934d3d834c90b078238f"}
    experimental_model
    Two-factor biotin/carnitine depletion and carnitine repletion in human HepG2 cells
    exposure
    Separate and combined biotin and carnitine depletion
    limitations
    This is a cell-culture demonstration of marker masking; it does not validate a diagnostic correction formula in pregnancy or the general population.
    nutrient_topic
    Biotin research collection; topical membership is not evidence of a direct dietary effect. · Biotin
    organism
    Homo sapiens
    plain_language
    Restoring carnitine exposed the unresolved biotin-related bottleneck.
    primary_references
    [b7-p25527659] In HepG2 cells, coexisting carnitine deficiency masks important indicators of marginal biotin deficiency. (2015). https://pubmed.ncbi.nlm.nih.gov/25527659/ DOI: 10.3945/jn.114.201343
    tissue_or_cell_type
    HepG2 hepatoma cells
    trigger_kind
    nutrient_deficiency Imported condition classification; unverified.

    Biotin: carboxylases, recycling, deficiency and nutrient interactions (2026-09-17) · lines 975–986

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Two-factor biotin/carnitine depletion and carnitine repletion in human HepG2 cells · source_derived_draft · unverified_draft

    ### b7-carnitine-unmasking Carnitine repletion of doubly deficient HepG2 cells increased acetylcarnitine, propionylcarnitine and 3-hydroxyisovalerylcarnitine each by more than 50-fold. Condition category: nutrient_deficiency nutrient_topic: Biotin research collection; topical membership is not evidence of a direct dietary effect. plain_language: Restoring carnitine exposed the unresolved biotin-related bottleneck. organism: Homo sapiens tissue_or_cell_type: HepG2 hepatoma cells experimental_model: Two-factor biotin/carnitine depletion and carnitine repletion in human HepG2 cells limitations: This is a cell-culture demonstration of marker masking; it does not validate a diagnostic correction formula in pregnancy or the general population. exposure: Separate and combined biotin and carnitine depletion evidence_span: {"source_cache": "artifacts/biotin-research/25527659.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "4a368eb2fa2020bb1cabc99b31a3420870a8256886d5934d3d834c90b078238f", "start_char": 0, "end_char": 2372, "text_sha256": "4a368eb2fa2020bb1cabc99b31a3420870a8256886d5934d3d834c90b078238f"} [b7-p25527659] In HepG2 cells, coexisting carnitine deficiency masks important indicators of marginal biotin deficiency. (2015). https://pubmed.ncbi.nlm.nih.gov/25527659/ DOI: 10.3945/jn.114.201343
    Complete structured claim and evidence

  4. Challenge-induced urinary 3-hydroxyisovalerylcarnitine rose more than twofold by day 14 in both biotin-depleted cohorts.

    L-Leucine → Human urinary 3-hydroxyisovalerylcarnitine excretion source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    nutrient_deficiency Imported condition classification; unverified.
    evidence_span
    {"source_cache": "artifacts/biotin-research/21918059.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "5cf1d7e25ae7bdfeb2ac3225cee8e4e660723d5197fc057f63327b3ad9eb3aaf", "start_char": 0, "end_char": 1723, "text_sha256": "5cf1d7e25ae7bdfeb2ac3225cee8e4e660723d5197fc057f63327b3ad9eb3aaf"}
    experimental_model
    Leucine challenge during biotin depletion in two cohorts of 5 and 7 healthy adults
    exposure
    28-day egg-white depletion; weekly oral leucine challenge
    limitations
    The challenge was sensitive but not diagnostically superior to 24-hour urine measurements; no validated universal threshold follows.
    nutrient_topic
    Biotin research collection; topical membership is not evidence of a direct dietary effect. · Biotin
    organism
    Homo sapiens
    plain_language
    The carnitine-linked side product also increased as biotin became limited.
    primary_references
    [b7-p21918059] Urinary excretion of 3-hydroxyisovaleric acid and 3-hydroxyisovaleryl carnitine increases in response to a leucine challenge in marginally biotin-deficient humans. (2011). https://pubmed.ncbi.nlm.nih.gov/21918059/ DOI: 10.3945/jn.111.146126
    tissue_or_cell_type
    Urine
    trigger_kind
    nutrient_deficiency Imported condition classification; unverified.

    Biotin: carboxylases, recycling, deficiency and nutrient interactions (2026-09-17) · lines 923–934

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Leucine challenge during biotin depletion in two cohorts of 5 and 7 healthy adults · source_derived_draft · unverified_draft

    ### b7-leucine-challenge-c5oh Challenge-induced urinary 3-hydroxyisovalerylcarnitine rose more than twofold by day 14 in both biotin-depleted cohorts. Condition category: nutrient_deficiency nutrient_topic: Biotin research collection; topical membership is not evidence of a direct dietary effect. plain_language: The carnitine-linked side product also increased as biotin became limited. organism: Homo sapiens tissue_or_cell_type: Urine experimental_model: Leucine challenge during biotin depletion in two cohorts of 5 and 7 healthy adults limitations: The challenge was sensitive but not diagnostically superior to 24-hour urine measurements; no validated universal threshold follows. exposure: 28-day egg-white depletion; weekly oral leucine challenge evidence_span: {"source_cache": "artifacts/biotin-research/21918059.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "5cf1d7e25ae7bdfeb2ac3225cee8e4e660723d5197fc057f63327b3ad9eb3aaf", "start_char": 0, "end_char": 1723, "text_sha256": "5cf1d7e25ae7bdfeb2ac3225cee8e4e660723d5197fc057f63327b3ad9eb3aaf"} [b7-p21918059] Urinary excretion of 3-hydroxyisovaleric acid and 3-hydroxyisovaleryl carnitine increases in response to a leucine challenge in marginally biotin-deficient humans. (2011). https://pubmed.ncbi.nlm.nih.gov/21918059/ DOI: 10.3945/jn.111.146126
    Complete structured claim and evidence

  5. Plasma 3-hydroxyisovalerylcarnitine was above the study reference interval in 7 of 10 adults by day 14 and 9 of 10 by day 28 of depletion, then fell with repletion.

    Biotin → Human plasma 3-hydroxyisovalerylcarnitine concentration source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    nutrient_deficiency Imported condition classification; unverified.
    evidence_span
    {"source_cache": "artifacts/biotin-research/20943794.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "1351a960b61efdce767a72da73d9ad8755e52f935e98b9510b4f68c2d5b83c9a", "start_char": 0, "end_char": 1877, "text_sha256": "1351a960b61efdce767a72da73d9ad8755e52f935e98b9510b4f68c2d5b83c9a"}
    experimental_model
    Depletion/repletion in 10 healthy adults, 8 women
    exposure
    28 days of undenatured egg white, followed by repletion
    limitations
    The study-specific reference interval and 10 participants do not define a universally diagnostic blood cutoff.
    nutrient_topic
    Biotin research collection; topical membership is not evidence of a direct dietary effect. · Biotin
    organism
    Homo sapiens
    plain_language
    A blood marker tracked depletion in most, but not all, participants.
    primary_references
    [b7-p20943794] Plasma concentration of 3-hydroxyisovaleryl carnitine is an early and sensitive indicator of marginal biotin deficiency in humans. (2010). https://pubmed.ncbi.nlm.nih.gov/20943794/ DOI: 10.3945/ajcn.110.002543
    tissue_or_cell_type
    Plasma
    trigger_kind
    nutrient_deficiency Imported condition classification; unverified.

    Biotin: carboxylases, recycling, deficiency and nutrient interactions (2026-09-17) · lines 936–947

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Depletion/repletion in 10 healthy adults, 8 women · source_derived_draft · unverified_draft

    ### b7-plasma-c5oh Plasma 3-hydroxyisovalerylcarnitine was above the study reference interval in 7 of 10 adults by day 14 and 9 of 10 by day 28 of depletion, then fell with repletion. Condition category: nutrient_deficiency nutrient_topic: Biotin research collection; topical membership is not evidence of a direct dietary effect. plain_language: A blood marker tracked depletion in most, but not all, participants. organism: Homo sapiens tissue_or_cell_type: Plasma experimental_model: Depletion/repletion in 10 healthy adults, 8 women limitations: The study-specific reference interval and 10 participants do not define a universally diagnostic blood cutoff. exposure: 28 days of undenatured egg white, followed by repletion evidence_span: {"source_cache": "artifacts/biotin-research/20943794.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "1351a960b61efdce767a72da73d9ad8755e52f935e98b9510b4f68c2d5b83c9a", "start_char": 0, "end_char": 1877, "text_sha256": "1351a960b61efdce767a72da73d9ad8755e52f935e98b9510b4f68c2d5b83c9a"} [b7-p20943794] Plasma concentration of 3-hydroxyisovaleryl carnitine is an early and sensitive indicator of marginal biotin deficiency in humans. (2010). https://pubmed.ncbi.nlm.nih.gov/20943794/ DOI: 10.3945/ajcn.110.002543
    Complete structured claim and evidence

  6. At the controlled intake, pregnant women excreted 69% more 3-HIA than controls, while urinary 3-HIA-carnitine was 27% lower.

    Biotin → Human urinary 3-hydroxyisovaleric acid excretion source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    biomarker_context Imported condition classification; unverified.
    evidence_span
    {"source_cache": "artifacts/biotin-research/25122647.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "6066b007a013c3c0c69336e7aba0e51baa39e3a24b6eb918cb4778e792376a9b", "start_char": 0, "end_char": 2354, "text_sha256": "6066b007a013c3c0c69336e7aba0e51baa39e3a24b6eb918cb4778e792376a9b"}
    experimental_model
    Controlled feeding: 26 pregnant, 28 lactating and 21 control women
    exposure
    57 micrograms dietary biotin per day for 10–12 weeks
    limitations
    Groups differed in reproductive state. Marker changes do not establish fetal outcomes, universal deficiency or a treatment dose.
    nutrient_topic
    Biotin research collection; topical membership is not evidence of a direct dietary effect. · Biotin
    organism
    Homo sapiens
    plain_language
    Two related markers moved in opposite directions during pregnancy.
    primary_references
    [b7-p25122647] Pregnancy and lactation alter biomarkers of biotin metabolism in women consuming a controlled diet. (2014). https://pubmed.ncbi.nlm.nih.gov/25122647/ DOI: 10.3945/jn.114.194472
    tissue_or_cell_type
    Urine and plasma
    trigger_kind
    biomarker_context Imported condition classification; unverified.

    Biotin: carboxylases, recycling, deficiency and nutrient interactions (2026-09-17) · lines 988–999

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Controlled feeding: 26 pregnant, 28 lactating and 21 control women · source_derived_draft · unverified_draft

    ### b7-pregnancy-3hia At the controlled intake, pregnant women excreted 69% more 3-HIA than controls, while urinary 3-HIA-carnitine was 27% lower. Condition category: biomarker_context nutrient_topic: Biotin research collection; topical membership is not evidence of a direct dietary effect. plain_language: Two related markers moved in opposite directions during pregnancy. organism: Homo sapiens tissue_or_cell_type: Urine and plasma experimental_model: Controlled feeding: 26 pregnant, 28 lactating and 21 control women limitations: Groups differed in reproductive state. Marker changes do not establish fetal outcomes, universal deficiency or a treatment dose. exposure: 57 micrograms dietary biotin per day for 10–12 weeks evidence_span: {"source_cache": "artifacts/biotin-research/25122647.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "6066b007a013c3c0c69336e7aba0e51baa39e3a24b6eb918cb4778e792376a9b", "start_char": 0, "end_char": 2354, "text_sha256": "6066b007a013c3c0c69336e7aba0e51baa39e3a24b6eb918cb4778e792376a9b"} [b7-p25122647] Pregnancy and lactation alter biomarkers of biotin metabolism in women consuming a controlled diet. (2014). https://pubmed.ncbi.nlm.nih.gov/25122647/ DOI: 10.3945/jn.114.194472
    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.

    Evidence, AI assistance and curation standards