Component

Tiglyl-CoA

Context-specific entity; species, compartment and exposure are stated on each 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. Human fibroblast and liver assays measured tiglyl-CoA hydration in the isoleucine branch; residual activity persisted despite ECHS1 deficiency, indicating overlapping hydratase capacity.

    Short-chain enoyl-CoA hydratase / ECHS1 → Tiglyl-CoA source_derived_draftungraded
    Experimental context and source evidence
    evidence_access
    Primary full text
    experimental_model
    Human ECHS1-deficient fibroblasts and liver; substrate-selective enzyme assays.
    limitations
    ECHS1 activity does not establish that it is the sole required isoleucine hydratase.
    nutrient_topic
    L-Isoleucine collection; species, compartment, exposure, co-substrates and manipulation remain explicit. · L-Isoleucine
    plain_language
    A reaction can have backup enzyme capacity in a tissue.
    primary_references
    Clinical and biochemical characterization of four patients with mutations in ECHS1. · 2015 · https://pubmed.ncbi.nlm.nih.gov/26081110/ · DOI 10.1186/s13023-015-0290-1

    L-Isoleucine: transport, translation, catabolism and cross-nutrient mechanisms (2026-09-19) · lines 194–200

    AI-assisted research curation; primary references, access levels and experimental limitations individually identified. Not publisher full text. · supports · Human ECHS1-deficient fibroblasts and liver; substrate-selective enzyme assays. · source_derived_draft · unverified_draft

    ## isoleucine-tiglyl-hydration A reaction can have backup enzyme capacity in a tissue. Human fibroblast and liver assays measured tiglyl-CoA hydration in the isoleucine branch; residual activity persisted despite ECHS1 deficiency, indicating overlapping hydratase capacity. Model: Human ECHS1-deficient fibroblasts and liver; substrate-selective enzyme assays. Limitations: ECHS1 activity does not establish that it is the sole required isoleucine hydratase. Evidence access: Primary full text Clinical and biochemical characterization of four patients with mutations in ECHS1. · 2015 · https://pubmed.ncbi.nlm.nih.gov/26081110/ · DOI 10.1186/s13023-015-0290-1
    Complete structured claim and evidence

Where it participates (unsigned role)

  1. Human ACADSB supported oxidation of 2-methylbutyryl-CoA; patient fibroblast assays and expression studies distinguished this isoleucine enzyme from valine-related ACAD8.

    Human short/branched-chain acyl-CoA dehydrogenase / ACADSB → 2-Methylbutyryl-CoA source_derived_draftungraded
    Experimental context and source evidence
    evidence_access
    Primary abstract
    experimental_model
    Human fibroblasts, sequence analysis and recombinant expression.
    limitations
    Does not make ACADSB and ACAD8 interchangeable.
    nutrient_topic
    L-Isoleucine collection; species, compartment, exposure, co-substrates and manipulation remain explicit. · L-Isoleucine
    plain_language
    Isoleucine and valine split into different enzyme routes after their shared early steps.
    primary_references
    Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: identification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism. · 2000 · https://pubmed.ncbi.nlm.nih.gov/11013134/ · DOI 10.1086/303105

    L-Isoleucine: transport, translation, catabolism and cross-nutrient mechanisms (2026-09-19) · lines 178–184

    AI-assisted research curation; primary references, access levels and experimental limitations individually identified. Not publisher full text. · supports · Human fibroblasts, sequence analysis and recombinant expression. · source_derived_draft · unverified_draft

    ## isoleucine-acadsb-reaction Isoleucine and valine split into different enzyme routes after their shared early steps. Human ACADSB supported oxidation of 2-methylbutyryl-CoA; patient fibroblast assays and expression studies distinguished this isoleucine enzyme from valine-related ACAD8. Model: Human fibroblasts, sequence analysis and recombinant expression. Limitations: Does not make ACADSB and ACAD8 interchangeable. Evidence access: Primary abstract Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: identification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism. · 2000 · https://pubmed.ncbi.nlm.nih.gov/11013134/ · DOI 10.1086/303105
    Complete structured claim and evidence

  2. Two HSD10 and four ACAT1 deficiency cases shared elevated 2-methyl-3-hydroxybutyrate/tiglylglycine patterns but had different clinical courses.

    Comparison of human HSD10 and ACAT1 deficiencies → Overlapping isoleucine-related urinary organic-acid pattern source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    machinery_impairment Imported condition classification; unverified.
    evidence_access
    Primary abstract
    experimental_model
    Six-patient human clinical and molecular study.
    limitations
    Small series; the suggested neurosteroid explanation was not experimentally proven by these comparisons.
    nutrient_topic
    L-Isoleucine collection; species, compartment, exposure, co-substrates and manipulation remain explicit. · L-Isoleucine
    plain_language
    Similar metabolite readings can arise from different machinery failures.
    primary_references
    Clinical and molecular analysis of 6 Chinese patients with isoleucine metabolism defects: identification of 3 novel mutations in the HSD17B10 and ACAT1 gene. · 2017 · https://pubmed.ncbi.nlm.nih.gov/28875337/ · DOI 10.1007/s11011-017-0097-y
    trigger_kind
    machinery_impairment Imported condition classification; unverified.

    L-Isoleucine: transport, translation, catabolism and cross-nutrient mechanisms (2026-09-19) · lines 250–256

    AI-assisted research curation; primary references, access levels and experimental limitations individually identified. Not publisher full text. · supports · Six-patient human clinical and molecular study. · source_derived_draft · unverified_draft

    ## isoleucine-catabolic-differential Similar metabolite readings can arise from different machinery failures. Two HSD10 and four ACAT1 deficiency cases shared elevated 2-methyl-3-hydroxybutyrate/tiglylglycine patterns but had different clinical courses. Model: Six-patient human clinical and molecular study. Limitations: Small series; the suggested neurosteroid explanation was not experimentally proven by these comparisons. Evidence access: Primary abstract Clinical and molecular analysis of 6 Chinese patients with isoleucine metabolism defects: identification of 3 novel mutations in the HSD17B10 and ACAT1 gene. · 2017 · https://pubmed.ncbi.nlm.nih.gov/28875337/ · DOI 10.1007/s11011-017-0097-y
    Complete structured claim and evidence

  3. ECHS1-deficient patients showed a dominant valine-metabolism defect rather than the expected isolated isoleucine block; liver retained tiglyl-CoA hydration capacity.

    Short-chain enoyl-CoA hydratase / ECHS1 → Tiglyl-CoA processing capacity in human tissues source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    machinery_impairment Imported condition classification; unverified.
    evidence_access
    Primary full text
    experimental_model
    Four-patient biochemical study with tissue enzyme comparisons.
    limitations
    Does not establish the same backup capacity in every cell type.
    nutrient_topic
    L-Isoleucine collection; species, compartment, exposure, co-substrates and manipulation remain explicit. · L-Isoleucine
    plain_language
    A pathway diagram alone can overstate an enzyme as an unavoidable gate.
    primary_references
    Clinical and biochemical characterization of four patients with mutations in ECHS1. · 2015 · https://pubmed.ncbi.nlm.nih.gov/26081110/ · DOI 10.1186/s13023-015-0290-1
    trigger_kind
    machinery_impairment Imported condition classification; unverified.

    L-Isoleucine: transport, translation, catabolism and cross-nutrient mechanisms (2026-09-19) · lines 202–208

    AI-assisted research curation; primary references, access levels and experimental limitations individually identified. Not publisher full text. · supports · Four-patient biochemical study with tissue enzyme comparisons. · source_derived_draft · unverified_draft

    ## isoleucine-echs1-boundary A pathway diagram alone can overstate an enzyme as an unavoidable gate. ECHS1-deficient patients showed a dominant valine-metabolism defect rather than the expected isolated isoleucine block; liver retained tiglyl-CoA hydration capacity. Model: Four-patient biochemical study with tissue enzyme comparisons. Limitations: Does not establish the same backup capacity in every cell type. Evidence access: Primary full text Clinical and biochemical characterization of four patients with mutations in ECHS1. · 2015 · https://pubmed.ncbi.nlm.nih.gov/26081110/ · DOI 10.1186/s13023-015-0290-1
    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