Component

Isoleucine shortage during human MSUD dietary treatment

Context-specific entity; species, compartment and exposure are stated on each 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.

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. A critically ill newborn with MSUD developed corneal epithelial loss with skin and intestinal symptoms during isolated isoleucine deficiency.

    Experimental context and source evidence
    availability_state
    nutrient_deficiency Imported condition classification; unverified.
    evidence_access
    Primary abstract
    experimental_model
    Single infant case during intensive metabolic dietary treatment.
    limitations
    Case attribution does not define a universal plasma threshold or the molecular epithelial mechanism.
    nutrient_topic
    L-Isoleucine collection; species, compartment, exposure, co-substrates and manipulation remain explicit. · L-Isoleucine
    plain_language
    Inadequate supply can affect more than muscle or growth.
    primary_references
    Corneal deepithelialization caused by acute deficiency of isoleucine during treatment of a patient with maple syrup urine disease. · 1996 · https://pubmed.ncbi.nlm.nih.gov/8741119/ · DOI 10.1111/j.1600-0420.1996.tb00386.x
    trigger_kind
    nutrient_deficiency Imported condition classification; unverified.

    L-Isoleucine: transport, translation, catabolism and cross-nutrient mechanisms (2026-09-19) · lines 330–336

    AI-assisted research curation; primary references, access levels and experimental limitations individually identified. Not publisher full text. · supports · Single infant case during intensive metabolic dietary treatment. · source_derived_draft · unverified_draft

    ## isoleucine-shortage-cornea Inadequate supply can affect more than muscle or growth. A critically ill newborn with MSUD developed corneal epithelial loss with skin and intestinal symptoms during isolated isoleucine deficiency. Model: Single infant case during intensive metabolic dietary treatment. Limitations: Case attribution does not define a universal plasma threshold or the molecular epithelial mechanism. Evidence access: Primary abstract Corneal deepithelialization caused by acute deficiency of isoleucine during treatment of a patient with maple syrup urine disease. · 1996 · https://pubmed.ncbi.nlm.nih.gov/8741119/ · DOI 10.1111/j.1600-0420.1996.tb00386.x
    Complete structured claim and evidence
  2. An infant on MSUD treatment developed dermatitis, diarrhea and hair loss associated with isoleucine deficiency; skin lesions resolved after combined dietary correction and topical treatment.

    Experimental context and source evidence
    availability_state
    nutrient_deficiency Imported condition classification; unverified.
    evidence_access
    Primary abstract
    experimental_model
    Human case report; isoleucine replacement, leucine restriction and topical mometasone used together.
    limitations
    Combined treatment prevents assigning the entire response to one intervention; not a general dermatitis diagnosis.
    nutrient_topic
    L-Isoleucine collection; species, compartment, exposure, co-substrates and manipulation remain explicit. · L-Isoleucine
    plain_language
    Restricting a breakdown substrate too far can damage tissues that still need it.
    primary_references
    Acrodermatitis dysmetabolica secondary to isoleucine deficiency in infant with maple syrup urine disease. · 2023 · https://pubmed.ncbi.nlm.nih.gov/38327590/ · DOI 10.4081/dr.2023.9750
    trigger_kind
    nutrient_deficiency Imported condition classification; unverified.

    L-Isoleucine: transport, translation, catabolism and cross-nutrient mechanisms (2026-09-19) · lines 322–328

    AI-assisted research curation; primary references, access levels and experimental limitations individually identified. Not publisher full text. · supports · Human case report; isoleucine replacement, leucine restriction and topical mometasone used together. · source_derived_draft · unverified_draft

    ## isoleucine-shortage-skin Restricting a breakdown substrate too far can damage tissues that still need it. An infant on MSUD treatment developed dermatitis, diarrhea and hair loss associated with isoleucine deficiency; skin lesions resolved after combined dietary correction and topical treatment. Model: Human case report; isoleucine replacement, leucine restriction and topical mometasone used together. Limitations: Combined treatment prevents assigning the entire response to one intervention; not a general dermatitis diagnosis. Evidence access: Primary abstract Acrodermatitis dysmetabolica secondary to isoleucine deficiency in infant with maple syrup urine disease. · 2023 · https://pubmed.ncbi.nlm.nih.gov/38327590/ · DOI 10.4081/dr.2023.9750
    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