EPEO4

Chr 5AR

aldehyde dehydrogenase 7 family member A1

Also known as: ATQ1, EPD, EPEO4, PDE

The protein encoded by this gene is an aldehyde dehydrogenase that detoxifies aldehydes from alcohol metabolism and lipid peroxidation, and participates in lysine catabolism in the mitochondrial matrix. Mutations cause early-onset, vitamin B6-dependent epilepsy (pyridoxine-dependent epilepsy) with autosomal recessive inheritance. This condition typically presents in the neonatal period with seizures that are refractory to standard anticonvulsants but respond to pyridoxine supplementation.

Summary from RefSeq, OMIM
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Primary Disease Associations & Inheritance

Epilepsy, early-onset, 4, vitamin B6-dependentMIM #266100
AR
0
Active trials
0
Pubs (1 yr)
0
P/LP submissions
P/LP missense
LOEUF
Mechanism
📖
GeneReview available — EPEO4
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/EPEO4?content-type=application/json&expand=1

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

EPEO4 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Investigation of epilepsy-related genes in a Drosophila model
Qu X et al.·Neural Regen Res
2024Functional
Top 1 full-text resultsSearch PubTator3 ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found