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HLD7

Chr 10AR

RNA polymerase III subunit A

Also known as: ADDH, C160, HLD7, RPC1, RPC155, WDRTS, hRPC155

This gene encodes the catalytic component of RNA polymerase III, which synthesizes small RNAs and acts as a sensor to detect foreign DNA and trigger innate immune responses. Biallelic mutations cause autosomal recessive hypomyelinating leukodystrophy 7, which may be associated with oligodontia and/or hypogonadotropic hypogonadism. The condition primarily affects white matter development in the brain along with potential involvement of dental and endocrine systems.

OMIMResearchSummary from RefSeq, OMIM
AR1 OMIM phenotype
Clinical SummaryHLD7
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/HLD7?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

HLD7 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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