POLR1C

Chr 6AR

RNA polymerase I and III subunit C

Also known as: AC40, HLD11, RPA39, RPA40, RPA5, RPAC1, RPC40, TCS3

The protein functions as a subunit of both RNA polymerase I and RNA polymerase III complexes, serving as part of the polymerase core element essential for ribosomal RNA and transfer RNA synthesis. Mutations cause autosomal recessive Treacher Collins syndrome 3 and hypomyelinating leukodystrophy 11. The pathogenic mechanism involves disruption of ribosomal biogenesis and cellular transcription processes.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.402 OMIM phenotypes
Clinical SummaryPOLR1C
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Gene-Disease Validity (ClinGen)
Treacher Collins syndrome 3 · ARModerate

Moderate evidence — consider for supplementary testing

2 total gene-disease associations curated

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.40LOEUF
pLI 0.000
Z-score 0.34
OE 0.91 (0.611.40)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-1.44Z-score
OE missense 1.29 (1.161.43)
252 obs / 195.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.91 (0.611.40)
00.351.4
Missense OE1.29 (1.161.43)
00.61.4
Synonymous OE1.24
01.21.6
LoF obs/exp: 15 / 16.5Missense obs/exp: 252 / 195.2Syn Z: -1.58

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

POLR1C · 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

No active trials found for this gene.

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Clinical Literature
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