Article · Wikipedia archive · Last revised Jul 10, 2026

POLR3A

RNA polymerase III subunit A is an enzyme which is encoded by the gene POLR3A.

Last revised
Jul 10, 2026
Read time
≈ 2 min
Length
456 w
Citations
14
Source
POLR3A
Identifiers
AliasesPOLR3A, ADDH, HLD7, RPC1, RPC155, hRPC155, polymerase (RNA) III subunit A, RNA polymerase III subunit A, WDRTS, C160
External IDsOMIM: 614258; MGI: 2681836; HomoloGene: 5124; GeneCards: POLR3A; OMA:POLR3A - orthologs
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_007055

NM_001081247
NM_183157

RefSeq (protein)

NP_008986

NP_001074716

Location (UCSC)Chr 10: 77.98 – 78.03 MbChr 14: 24.5 – 24.54 Mb
PubMed search34
Wikidata
View/Edit HumanView/Edit Mouse

RNA polymerase III subunit A is an enzyme which is encoded by the gene POLR3A.5

Gene

The POLR3A gene is located on the long arm (q) of chromosome 10 on position 22.3, from base pair from base pair 77,975,149 to base pair 78,029,515.6

Function

RNA polymerase III subunit A (this gene) along with subunit B (POLR3B) forms the catalytic core of RNA polymerase III. Together, these two subunits create the active center of the enzyme where RNA synthesis occurs.7

RNA polymerase III subunit A also detects foreign DNA and triggers an innate immune response.8

Clinical significance

Wiedemann–Rautenstrauch syndrome

Wiedemann–Rautenstrauch syndrome is a rare genetic disorder, which is characterised by prenatal and postnatal growth restriction, lipodystrophy, sparse hair, relatively large head, and a peculiar face. Intellectual disability is also common.9

4H leukodystrophy

4H leukodystrophy is a rare genetic disorder, which is characterised by hypogonadotropic hypogonadism, dental abnormalities, and hypomyelination.10

References

References

  1. GRCh38: Ensembl release 89: ENSG00000148606Ensembl, May 2017
  2. GRCm38: Ensembl release 89: ENSMUSG00000025280Ensembl, May 2017
  3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. "POLR3A Gene - RNA Polymerase III Subunit A".
  6. "Genome Data Viewer - NCBI". www.ncbi.nlm.nih.gov. Retrieved 2025-07-29.
  7. Saitsu H, Osaka H, Sasaki M, Takanashi JI, Hamada K, Yamashita A, et al. (2011-11-11). "Mutations in POLR3A and POLR3B Encoding RNA Polymerase III Subunits Cause an Autosomal-Recessive Hypomyelinating Leukoencephalopathy". American Journal of Human Genetics. 89 (5): 644–651. doi:10.1016/j.ajhg.2011.10.003. ISSN 0002-9297. PMC 3213392. PMID 22036171.
  8. "POLR3A RNA polymerase III subunit A [Homo sapiens (human)] - Gene - NCBI". www.ncbi.nlm.nih.gov. Retrieved 2025-07-29.
  9. "Orphanet: Wiedemann-Rautenstrauch syndrome". www.orpha.net. Retrieved 2025-07-29.
  10. "Orphanet: 4H leukodystrophy". www.orpha.net. Retrieved 2025-07-29.