Component

Human biotinidase / BTD

Human biotinidase / BTD. Species, exposure and limitations are retained in each linked claim.

5 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 it acts on

  1. Biotinidase impairment reduced carboxylase biotinylation and disrupted HLCS and carboxylase expression in the studied human cell model.

    Human biotinidase / BTD → Human HLCS expression source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    machinery_impairment Imported condition classification; unverified.
    evidence_span
    {"source_cache": "artifacts/biotin-research/18845537.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "86a05dc5e8386be649b3f876891503e507217c8f2dd11d9637c67e76effda407", "start_char": 0, "end_char": 1458, "text_sha256": "86a05dc5e8386be649b3f876891503e507217c8f2dd11d9637c67e76effda407"}
    experimental_model
    Biotinidase-deficient human cell model compared with control cells
    exposure
    Biotin recycling impairment and biotin repletion experiments
    limitations
    The proposed brain-protective tissue-allocation mechanism remains an author model, not an established human ranking.
    nutrient_topic
    Biotin research collection; topical membership is not evidence of a direct dietary effect. · Biotin
    organism
    Homo sapiens
    plain_language
    Loss of recycling can affect both cofactor attachment and expression of the attachment machinery.
    primary_references
    [b7-p18845537] Impaired biotinidase activity disrupts holocarboxylase synthetase expression in late onset multiple carboxylase deficiency. (2008). https://pubmed.ncbi.nlm.nih.gov/18845537/ DOI: 10.1074/jbc.m806985200
    tissue_or_cell_type
    Cultured patient-derived cells
    trigger_kind
    machinery_impairment Imported condition classification; unverified.

    Biotin: carboxylases, recycling, deficiency and nutrient interactions (2026-09-17) · lines 481–492

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Biotinidase-deficient human cell model compared with control cells · source_derived_draft · unverified_draft

    ### b7-btd-expression Biotinidase impairment reduced carboxylase biotinylation and disrupted HLCS and carboxylase expression in the studied human cell model. Condition category: machinery_impairment nutrient_topic: Biotin research collection; topical membership is not evidence of a direct dietary effect. plain_language: Loss of recycling can affect both cofactor attachment and expression of the attachment machinery. organism: Homo sapiens tissue_or_cell_type: Cultured patient-derived cells experimental_model: Biotinidase-deficient human cell model compared with control cells limitations: The proposed brain-protective tissue-allocation mechanism remains an author model, not an established human ranking. exposure: Biotin recycling impairment and biotin repletion experiments evidence_span: {"source_cache": "artifacts/biotin-research/18845537.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "86a05dc5e8386be649b3f876891503e507217c8f2dd11d9637c67e76effda407", "start_char": 0, "end_char": 1458, "text_sha256": "86a05dc5e8386be649b3f876891503e507217c8f2dd11d9637c67e76effda407"} [b7-p18845537] Impaired biotinidase activity disrupts holocarboxylase synthetase expression in late onset multiple carboxylase deficiency. (2008). https://pubmed.ncbi.nlm.nih.gov/18845537/ DOI: 10.1074/jbc.m806985200
    Complete structured claim and evidence

  2. The five affected children had serum biotinidase activity at 0–3% of the control mean, with combined carboxylase abnormalities.

    Human biotinidase / BTD → Human serum biotinidase activity source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    machinery_impairment Imported condition classification; unverified.
    evidence_span
    {"source_cache": "artifacts/biotin-research/6883721.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "0f881dd651d45537377661fc515239c3babb7160c3dbd53b8b0611ba33625aaa", "start_char": 0, "end_char": 1500, "text_sha256": "0f881dd651d45537377661fc515239c3babb7160c3dbd53b8b0611ba33625aaa"}
    experimental_model
    Serum enzyme assays in 5 children with late-onset multiple carboxylase deficiency and family members
    exposure
    Inherited deficiency; patient/control comparison
    limitations
    Early small case series; the serum enzyme phenotype is genetic machinery impairment, not proof of low dietary intake.
    nutrient_topic
    Biotin research collection; topical membership is not evidence of a direct dietary effect. · Biotin
    organism
    Homo sapiens
    plain_language
    A recycling defect can affect several enzyme pathways at once.
    primary_references
    [b7-p6883721] Biotinidase deficiency: the enzymatic defect in late-onset multiple carboxylase deficiency. (1983). https://pubmed.ncbi.nlm.nih.gov/6883721/ DOI: 10.1016/0009-8981(83)90096-7
    tissue_or_cell_type
    Serum and biochemical disease phenotype
    trigger_kind
    machinery_impairment Imported condition classification; unverified.

    Biotin: carboxylases, recycling, deficiency and nutrient interactions (2026-09-17) · lines 468–479

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Serum enzyme assays in 5 children with late-onset multiple carboxylase deficiency and family members · source_derived_draft · unverified_draft

    ### b7-btd-low-activity The five affected children had serum biotinidase activity at 0–3% of the control mean, with combined carboxylase abnormalities. Condition category: machinery_impairment nutrient_topic: Biotin research collection; topical membership is not evidence of a direct dietary effect. plain_language: A recycling defect can affect several enzyme pathways at once. organism: Homo sapiens tissue_or_cell_type: Serum and biochemical disease phenotype experimental_model: Serum enzyme assays in 5 children with late-onset multiple carboxylase deficiency and family members limitations: Early small case series; the serum enzyme phenotype is genetic machinery impairment, not proof of low dietary intake. exposure: Inherited deficiency; patient/control comparison evidence_span: {"source_cache": "artifacts/biotin-research/6883721.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "0f881dd651d45537377661fc515239c3babb7160c3dbd53b8b0611ba33625aaa", "start_char": 0, "end_char": 1500, "text_sha256": "0f881dd651d45537377661fc515239c3babb7160c3dbd53b8b0611ba33625aaa"} [b7-p6883721] Biotinidase deficiency: the enzymatic defect in late-onset multiple carboxylase deficiency. (1983). https://pubmed.ncbi.nlm.nih.gov/6883721/ DOI: 10.1016/0009-8981(83)90096-7
    Complete structured claim and evidence

  3. Biotinidase releases biotin from its linkage to the epsilon-amino group of lysine, allowing the cofactor to be reused.

    Human biotinidase / BTD → Biocytin / N-epsilon-biotinyl-L-lysine source_derived_draftungraded
    Experimental context and source evidence
    evidence_span
    {"source_cache": "artifacts/biotin-research/6883721.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "0f881dd651d45537377661fc515239c3babb7160c3dbd53b8b0611ba33625aaa", "start_char": 0, "end_char": 1500, "text_sha256": "0f881dd651d45537377661fc515239c3babb7160c3dbd53b8b0611ba33625aaa"}
    experimental_model
    Serum enzyme assays in 5 children with late-onset multiple carboxylase deficiency and family members
    exposure
    Inherited deficiency; patient/control comparison
    limitations
    Early small case series; the serum enzyme phenotype is genetic machinery impairment, not proof of low dietary intake.
    nutrient_topic
    Biotin research collection; topical membership is not evidence of a direct dietary effect. · Biotin
    organism
    Homo sapiens
    plain_language
    Biotinidase recovers biotin after biotin-containing material is broken down.
    primary_references
    [b7-p6883721] Biotinidase deficiency: the enzymatic defect in late-onset multiple carboxylase deficiency. (1983). https://pubmed.ncbi.nlm.nih.gov/6883721/ DOI: 10.1016/0009-8981(83)90096-7
    tissue_or_cell_type
    Serum and biochemical disease phenotype

    Biotin: carboxylases, recycling, deficiency and nutrient interactions (2026-09-17) · lines 455–466

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Serum enzyme assays in 5 children with late-onset multiple carboxylase deficiency and family members · source_derived_draft · unverified_draft

    ### b7-btd-recycling Biotinidase releases biotin from its linkage to the epsilon-amino group of lysine, allowing the cofactor to be reused. Condition category: normal nutrient_topic: Biotin research collection; topical membership is not evidence of a direct dietary effect. plain_language: Biotinidase recovers biotin after biotin-containing material is broken down. organism: Homo sapiens tissue_or_cell_type: Serum and biochemical disease phenotype experimental_model: Serum enzyme assays in 5 children with late-onset multiple carboxylase deficiency and family members limitations: Early small case series; the serum enzyme phenotype is genetic machinery impairment, not proof of low dietary intake. exposure: Inherited deficiency; patient/control comparison evidence_span: {"source_cache": "artifacts/biotin-research/6883721.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "0f881dd651d45537377661fc515239c3babb7160c3dbd53b8b0611ba33625aaa", "start_char": 0, "end_char": 1500, "text_sha256": "0f881dd651d45537377661fc515239c3babb7160c3dbd53b8b0611ba33625aaa"} [b7-p6883721] Biotinidase deficiency: the enzymatic defect in late-onset multiple carboxylase deficiency. (1983). https://pubmed.ncbi.nlm.nih.gov/6883721/ DOI: 10.1016/0009-8981(83)90096-7
    Complete structured claim and evidence

Where it participates (unsigned role)

  1. Newborn-screened children with biotinidase deficiency treated from early life and with good adherence generally had normal physical and cognitive development.

    Biotin → Clinical outcome of human biotinidase deficiency source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    machinery_impairment Imported condition classification; unverified.
    evidence_span
    {"source_cache": "artifacts/biotin-research/25144890.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "462a23bfc98686c02d0d6d92d923de7e05f646daa2e90a3373bde6a251e75f94", "start_char": 0, "end_char": 1414, "text_sha256": "462a23bfc98686c02d0d6d92d923de7e05f646daa2e90a3373bde6a251e75f94"}
    experimental_model
    Follow-up of 142 newborn-screened children, 22 profound and 120 partial biotinidase deficiency
    exposure
    Biotin treatment initiated soon after birth
    limitations
    Observational treated cohort, not a randomized trial or evidence for supplementation of biotin-replete people.
    nutrient_topic
    Biotin research collection; topical membership is not evidence of a direct dietary effect. · Biotin
    organism
    Homo sapiens
    plain_language
    Early identification and treatment of a recycling defect can prevent much of its harm.
    primary_references
    [b7-p25144890] Outcomes of individuals with profound and partial biotinidase deficiency ascertained by newborn screening in Michigan over 25 years. (2015). https://pubmed.ncbi.nlm.nih.gov/25144890/ DOI: 10.1038/gim.2014.104
    tissue_or_cell_type
    Clinical development
    trigger_kind
    machinery_impairment Imported condition classification; unverified.

    Biotin: carboxylases, recycling, deficiency and nutrient interactions (2026-09-17) · lines 533–544

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Follow-up of 142 newborn-screened children, 22 profound and 120 partial biotinidase deficiency · source_derived_draft · unverified_draft

    ### b7-btd-early-treatment Newborn-screened children with biotinidase deficiency treated from early life and with good adherence generally had normal physical and cognitive development. Condition category: machinery_impairment nutrient_topic: Biotin research collection; topical membership is not evidence of a direct dietary effect. plain_language: Early identification and treatment of a recycling defect can prevent much of its harm. organism: Homo sapiens tissue_or_cell_type: Clinical development experimental_model: Follow-up of 142 newborn-screened children, 22 profound and 120 partial biotinidase deficiency limitations: Observational treated cohort, not a randomized trial or evidence for supplementation of biotin-replete people. exposure: Biotin treatment initiated soon after birth evidence_span: {"source_cache": "artifacts/biotin-research/25144890.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "462a23bfc98686c02d0d6d92d923de7e05f646daa2e90a3373bde6a251e75f94", "start_char": 0, "end_char": 1414, "text_sha256": "462a23bfc98686c02d0d6d92d923de7e05f646daa2e90a3373bde6a251e75f94"} [b7-p25144890] Outcomes of individuals with profound and partial biotinidase deficiency ascertained by newborn screening in Michigan over 25 years. (2015). https://pubmed.ncbi.nlm.nih.gov/25144890/ DOI: 10.1038/gim.2014.104
    Complete structured claim and evidence

  2. The 2004 study identified H4 K8/K12 as candidate biotinylation sites in peptide assays and reported K12-biotinylated H4 immunoreactivity in human nuclear extracts.

    Biotin → Human histone H4 K12 biotinylation reported in selected assays source_derived_draftungraded
    Experimental context and source evidence
    evidence_span
    {"source_cache": "artifacts/biotin-research/15153116.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "a3297d283f8a3980a77f1ba1d53f326fb32ee3971c76bda71ef9ba235fb53965", "start_char": 0, "end_char": 1762, "text_sha256": "a3297d283f8a3980a77f1ba1d53f326fb32ee3971c76bda71ef9ba235fb53965"}
    experimental_model
    Synthetic human histone H4 peptide biotinylation and human nuclear-extract immunodetection
    exposure
    Biotinidase incubation, residue substitutions and antibody/streptavidin detection
    limitations
    Peptide reactions and immunoreactivity do not establish abundant endogenous histone biotinylation; later assay-method work explicitly challenges this interpretation.
    nutrient_topic
    Biotin research collection; topical membership is not evidence of a direct dietary effect. · Biotin
    organism
    Homo sapiens
    plain_language
    Early experiments proposed that biotin could also mark histones.
    primary_references
    [b7-p15153116] K8 and K12 are biotinylated in human histone H4. (2004). https://pubmed.ncbi.nlm.nih.gov/15153116/ DOI: 10.1111/j.1432-1033.2004.04167.x
    tissue_or_cell_type
    Synthetic H4 peptides and human cell extracts

    Biotin: carboxylases, recycling, deficiency and nutrient interactions (2026-09-17) · lines 1157–1168

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Synthetic human histone H4 peptide biotinylation and human nuclear-extract immunodetection · source_derived_draft · unverified_draft

    ### b7-histone-h4-report The 2004 study identified H4 K8/K12 as candidate biotinylation sites in peptide assays and reported K12-biotinylated H4 immunoreactivity in human nuclear extracts. Condition category: normal nutrient_topic: Biotin research collection; topical membership is not evidence of a direct dietary effect. plain_language: Early experiments proposed that biotin could also mark histones. organism: Homo sapiens tissue_or_cell_type: Synthetic H4 peptides and human cell extracts experimental_model: Synthetic human histone H4 peptide biotinylation and human nuclear-extract immunodetection limitations: Peptide reactions and immunoreactivity do not establish abundant endogenous histone biotinylation; later assay-method work explicitly challenges this interpretation. exposure: Biotinidase incubation, residue substitutions and antibody/streptavidin detection evidence_span: {"source_cache": "artifacts/biotin-research/15153116.abstract.txt", "locator": "Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets", "file_sha256": "a3297d283f8a3980a77f1ba1d53f326fb32ee3971c76bda71ef9ba235fb53965", "start_char": 0, "end_char": 1762, "text_sha256": "a3297d283f8a3980a77f1ba1d53f326fb32ee3971c76bda71ef9ba235fb53965"} [b7-p15153116] K8 and K12 are biotinylated in human histone H4. (2004). https://pubmed.ncbi.nlm.nih.gov/15153116/ DOI: 10.1111/j.1432-1033.2004.04167.x
    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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