Component

alpha-Aminoadipate semialdehyde

Independent small molecule record; interpretation is limited by each linked claim and its study context.

7 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. Alpha-aminoadipate semialdehyde interconverts with its cyclic Schiff base P6C.

    alpha-Aminoadipate semialdehyde → Delta1-piperideine-6-carboxylate source_derived_draftungraded
    Experimental context and source evidence
    experimental_model
    Biochemical characterization
    limitations
    The two chemical species are separate entities connected by equilibrium.
    organism
    Homo sapiens
    plain_language
    One intermediate exists in open-chain and ring forms.
    primary_references
    [mills2006] Mutations in antiquitin in individuals with pyridoxine-dependent seizures. (2006). https://pubmed.ncbi.nlm.nih.gov/16491085/ DOI: 10.1038/nm1366
    tissue_or_cell_type
    Lysine-catabolizing compartments and body fluids

    L-Lysine: mechanism-first literature curation (2026-09-17) · lines 94–102

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Biochemical characterization · source_derived_draft · unverified_draft

    ### aasa-p6c-equilibrium Alpha-aminoadipate semialdehyde interconverts with its cyclic Schiff base P6C. Plain language: One intermediate exists in open-chain and ring forms. Condition category: normal organism: Homo sapiens tissue_or_cell_type: Lysine-catabolizing compartments and body fluids experimental_model: Biochemical characterization limitations: The two chemical species are separate entities connected by equilibrium. [mills2006] Mutations in antiquitin in individuals with pyridoxine-dependent seizures. (2006). https://pubmed.ncbi.nlm.nih.gov/16491085/ DOI: 10.1038/nm1366
    Complete structured claim and evidence

  2. Elevated AASA was observed in an 18-patient PDE cohort with ALDH7A1 investigation, including during pyridoxine treatment.

    alpha-Aminoadipate semialdehyde → Antiquitin / ALDH7A1 source_derived_draftungraded
    Experimental context and source evidence
    affected_machinery
    ALDH7A1 under investigation
    availability_state
    biomarker_context Imported condition classification; unverified.
    deficiency_not_equivalent
    Dietary lysine deficiency
    experimental_model
    Human clinical cohort; plasma and urine assays
    limitations
    A biomarker is not itself a diagnosis or proof of dietary lysine deficiency.
    organism
    Homo sapiens
    plain_language
    AASA measurement can reveal disturbed lysine breakdown.
    primary_references
    [plecko2007] Biochemical and molecular characterization of 18 patients with pyridoxine-dependent epilepsy and mutations of the antiquitin (ALDH7A1) gene (2007). https://pubmed.ncbi.nlm.nih.gov/17068770/ DOI: 10.1002/humu.20433
    tissue_or_cell_type
    Plasma and urine
    trigger_kind
    biomarker_context Imported condition classification; unverified.

    L-Lysine: mechanism-first literature curation (2026-09-17) · lines 324–334

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Human clinical cohort; plasma and urine assays · source_derived_draft · unverified_draft

    ### aasa-pde-biomarker Elevated AASA was observed in an 18-patient PDE cohort with ALDH7A1 investigation, including during pyridoxine treatment. Plain language: AASA measurement can reveal disturbed lysine breakdown. Condition category: biomarker_context organism: Homo sapiens tissue_or_cell_type: Plasma and urine experimental_model: Human clinical cohort; plasma and urine assays limitations: A biomarker is not itself a diagnosis or proof of dietary lysine deficiency. affected_machinery: ALDH7A1 under investigation deficiency_not_equivalent: Dietary lysine deficiency [plecko2007] Biochemical and molecular characterization of 18 patients with pyridoxine-dependent epilepsy and mutations of the antiquitin (ALDH7A1) gene (2007). https://pubmed.ncbi.nlm.nih.gov/17068770/ DOI: 10.1002/humu.20433
    Complete structured claim and evidence

  3. ALDH7A1 oxidizes alpha-aminoadipate semialdehyde to alpha-aminoadipate using NAD+.

    alpha-Aminoadipate semialdehyde → L-alpha-Aminoadipate source_derived_draftungraded
    Experimental context and source evidence
    experimental_model
    Human ALDH7A1 biochemical and genetic study
    limitations
    Compartmental isoform distribution is not resolved by this claim.
    organism
    Homo sapiens
    plain_language
    Antiquitin clears the aldehyde intermediate.
    primary_references
    [luo2015] Structural Basis of Substrate Recognition by Aldehyde Dehydrogenase 7A1 (2015). https://pubmed.ncbi.nlm.nih.gov/26260980/ DOI: 10.1021/acs.biochem.5b00754
    tissue_or_cell_type
    Lysine-catabolizing tissues

    L-Lysine: mechanism-first literature curation (2026-09-17) · lines 104–112

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Human ALDH7A1 biochemical and genetic study · source_derived_draft · unverified_draft

    ### aldh7a1-oxidation ALDH7A1 oxidizes alpha-aminoadipate semialdehyde to alpha-aminoadipate using NAD+. Plain language: Antiquitin clears the aldehyde intermediate. Condition category: normal organism: Homo sapiens tissue_or_cell_type: Lysine-catabolizing tissues experimental_model: Human ALDH7A1 biochemical and genetic study limitations: Compartmental isoform distribution is not resolved by this claim. [luo2015] Structural Basis of Substrate Recognition by Aldehyde Dehydrogenase 7A1 (2015). https://pubmed.ncbi.nlm.nih.gov/26260980/ DOI: 10.1021/acs.biochem.5b00754
    Complete structured claim and evidence

What acts on it

  1. The AASS dehydrogenase domain oxidatively cleaves saccharopine, producing alpha-aminoadipate semialdehyde and glutamate with NAD+ reduction.

    Saccharopine → alpha-Aminoadipate semialdehyde source_derived_draftungraded
    Experimental context and source evidence
    experimental_model
    Human AASS cloning, localization and familial hyperlysinemia genetics
    limitations
    Biochemical capacity does not quantify flux in every human tissue.
    organism
    Homo sapiens
    plain_language
    The second AASS activity opens the next lysine breakdown step.
    primary_references
    [sacksteder2000] Identification of the alpha-aminoadipic semialdehyde synthase gene, which is defective in familial hyperlysinemia (2000). https://pmc.ncbi.nlm.nih.gov/articles/PMC1378037/ DOI: 10.1086/302919
    tissue_or_cell_type
    Mitochondrial matrix

    L-Lysine: mechanism-first literature curation (2026-09-17) · lines 84–92

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Human AASS cloning, localization and familial hyperlysinemia genetics · source_derived_draft · unverified_draft

    ### aass-saccharopine-dehydrogenase The AASS dehydrogenase domain oxidatively cleaves saccharopine, producing alpha-aminoadipate semialdehyde and glutamate with NAD+ reduction. Plain language: The second AASS activity opens the next lysine breakdown step. Condition category: normal organism: Homo sapiens tissue_or_cell_type: Mitochondrial matrix experimental_model: Human AASS cloning, localization and familial hyperlysinemia genetics limitations: Biochemical capacity does not quantify flux in every human tissue. [sacksteder2000] Identification of the alpha-aminoadipic semialdehyde synthase gene, which is defective in familial hyperlysinemia (2000). https://pmc.ncbi.nlm.nih.gov/articles/PMC1378037/ DOI: 10.1086/302919
    Complete structured claim and evidence

  2. ALDH7A1 deficiency reduces aldehyde clearance, increasing AASA and its equilibrium partner P6C.

    Antiquitin / ALDH7A1 → alpha-Aminoadipate semialdehyde source_derived_draftungraded
    Experimental context and source evidence
    affected_machinery
    ALDH7A1
    availability_state
    machinery_impairment Imported condition classification; unverified.
    deficiency_not_equivalent
    Dietary lysine deficiency
    experimental_model
    Human patient biochemical and genetic evidence
    limitations
    Inherited enzyme failure is distinct from inadequate lysine intake.
    organism
    Homo sapiens
    plain_language
    An enzyme block allows reactive lysine metabolites to accumulate.
    primary_references
    [mills2006] Mutations in antiquitin in individuals with pyridoxine-dependent seizures. (2006). https://pubmed.ncbi.nlm.nih.gov/16491085/ DOI: 10.1038/nm1366
    tissue_or_cell_type
    Affected tissues and body fluids
    trigger_kind
    machinery_impairment Imported condition classification; unverified.

    L-Lysine: mechanism-first literature curation (2026-09-17) · lines 254–264

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Human patient biochemical and genetic evidence · source_derived_draft · unverified_draft

    ### aldh7a1-metabolite-accumulation ALDH7A1 deficiency reduces aldehyde clearance, increasing AASA and its equilibrium partner P6C. Plain language: An enzyme block allows reactive lysine metabolites to accumulate. Condition category: machinery_impairment organism: Homo sapiens tissue_or_cell_type: Affected tissues and body fluids experimental_model: Human patient biochemical and genetic evidence limitations: Inherited enzyme failure is distinct from inadequate lysine intake. affected_machinery: ALDH7A1 deficiency_not_equivalent: Dietary lysine deficiency [mills2006] Mutations in antiquitin in individuals with pyridoxine-dependent seizures. (2006). https://pubmed.ncbi.nlm.nih.gov/16491085/ DOI: 10.1038/nm1366
    Complete structured claim and evidence

Where it participates (unsigned role)

  1. The antiquitin defect was linked to accumulation of alpha-AASA/P6C in the lysine degradation pathway.

    Biallelic ALDH7A1 disease genotypes studied by Mills 2006 → Delta1-piperideine-6-carboxylate source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    machinery_impairment Imported condition classification; unverified.
    cross_nutrient
    Lysine catabolism generates the metabolite that can sequester PLP.
    experimental_model
    Affected children, expressed human ALDH7A1 variants and metabolite chemistry
    exposure
    ALDH7A1-associated disease and metabolite analysis.
    limitations
    This is inherited pathway failure, not lysine toxicity in healthy people.
    nutrient_topic
    Vitamin B6 research collection; topical membership is not evidence of a direct dietary effect. · Vitamin B6
    organism
    Homo sapiens
    plain_language
    A blocked lysine pathway builds up B6-reactive metabolites.
    primary_references
    [mills-2006-aldh7a1] Mutations in antiquitin in individuals with pyridoxine-dependent seizures (2006). https://doi.org/10.1038/nm1366 DOI: 10.1038/nm1366
    tissue_or_cell_type
    Patient biological samples and biochemical pathway analysis
    trigger_kind
    machinery_impairment Imported condition classification; unverified.

    Vitamin B6: mechanisms, deficiency and nutrient interactions (2026-09-17) · lines 1158–1169

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Affected children, expressed human ALDH7A1 variants and metabolite chemistry · source_derived_draft · unverified_draft

    ### b6-neuro-aldh7a1-metabolite-accumulation The antiquitin defect was linked to accumulation of alpha-AASA/P6C in the lysine degradation pathway. Condition category: machinery_impairment nutrient_topic: Vitamin B6 research collection; topical membership is not evidence of a direct dietary effect. plain_language: A blocked lysine pathway builds up B6-reactive metabolites. organism: Homo sapiens tissue_or_cell_type: Patient biological samples and biochemical pathway analysis experimental_model: Affected children, expressed human ALDH7A1 variants and metabolite chemistry limitations: This is inherited pathway failure, not lysine toxicity in healthy people. exposure: ALDH7A1-associated disease and metabolite analysis. cross_nutrient: Lysine catabolism generates the metabolite that can sequester PLP. [mills-2006-aldh7a1] Mutations in antiquitin in individuals with pyridoxine-dependent seizures (2006). https://doi.org/10.1038/nm1366 DOI: 10.1038/nm1366
    Complete structured claim and evidence

  2. ALDH7A1 variants identified in affected children abolished antiquitin activity in the reported functional assays.

    Biallelic ALDH7A1 disease genotypes studied by Mills 2006 → Antiquitin / ALDH7A1 source_derived_draftungraded
    Experimental context and source evidence
    availability_state
    machinery_impairment Imported condition classification; unverified.
    cross_nutrient
    Lysine catabolic machinery can impair intracellular B6 availability.
    experimental_model
    Affected children, expressed human ALDH7A1 variants and metabolite chemistry
    exposure
    Disease-variant functional expression.
    limitations
    Applies to the studied variants; not all possible ALDH7A1 alleles.
    nutrient_topic
    Vitamin B6 research collection; topical membership is not evidence of a direct dietary effect. · Vitamin B6
    organism
    Homo sapiens
    plain_language
    The inherited defect blocks a lysine-breakdown enzyme.
    primary_references
    [mills-2006-aldh7a1] Mutations in antiquitin in individuals with pyridoxine-dependent seizures (2006). https://doi.org/10.1038/nm1366 DOI: 10.1038/nm1366
    tissue_or_cell_type
    Patient-linked expression experiments
    trigger_kind
    machinery_impairment Imported condition classification; unverified.

    Vitamin B6: mechanisms, deficiency and nutrient interactions (2026-09-17) · lines 1145–1156

    AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text. · supports · Affected children, expressed human ALDH7A1 variants and metabolite chemistry · source_derived_draft · unverified_draft

    ### b6-neuro-aldh7a1-variants ALDH7A1 variants identified in affected children abolished antiquitin activity in the reported functional assays. Condition category: machinery_impairment nutrient_topic: Vitamin B6 research collection; topical membership is not evidence of a direct dietary effect. plain_language: The inherited defect blocks a lysine-breakdown enzyme. organism: Homo sapiens tissue_or_cell_type: Patient-linked expression experiments experimental_model: Affected children, expressed human ALDH7A1 variants and metabolite chemistry limitations: Applies to the studied variants; not all possible ALDH7A1 alleles. exposure: Disease-variant functional expression. cross_nutrient: Lysine catabolic machinery can impair intracellular B6 availability. [mills-2006-aldh7a1] Mutations in antiquitin in individuals with pyridoxine-dependent seizures (2006). https://doi.org/10.1038/nm1366 DOI: 10.1038/nm1366
    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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