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DRTA3

Chr 7AR

ATPase H+ transporting V0 subunit a4

Also known as: A4, ATP6N1B, ATP6N2, DRTA3, RDRTA2, RTA1C, RTADR, STV1

This gene encodes the a4 subunit of vacuolar ATPase (V-ATPase), a multisubunit enzyme that acidifies intracellular compartments and is essential for protein sorting, endocytosis, and synaptic vesicle function. Biallelic mutations cause autosomal recessive distal renal tubular acidosis type 3, which may occur with or without sensorineural hearing loss. This condition affects the kidney's ability to acidify urine and can present with failure to thrive, nephrolithiasis, and nephrocalcinosis in infancy or childhood.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM
AR1 OMIM phenotype
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GeneReview available — DRTA3
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/DRTA3?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

DRTA3 · 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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No publications found for DRTA3