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MTDPS4A

Chr 15AR

DNA polymerase gamma, catalytic subunit

Also known as: MIRAS, MTDPS4A, MTDPS4B, PEO, POLG1, POLGA, PolG-alpha, SANDO

The encoded protein is the catalytic subunit of mitochondrial DNA polymerase, which replicates mitochondrial DNA. Mutations cause mitochondrial DNA depletion syndrome 4A (Alpers type), characterized by progressive neurodegeneration, hepatopathy, and seizures typically presenting in infancy or early childhood. This condition follows autosomal recessive inheritance.

OMIMResearchSummary from RefSeq, OMIM
AR1 OMIM phenotype
Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/MTDPS4A?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

MTDPS4A · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

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Clinical Literature
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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

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