POLR3F

Chr 20AD

RNA polymerase III subunit F

Also known as: C34, IMD101, RPC39, RPC6

POLR3F encodes a subunit of RNA polymerase III that transcribes small non-coding RNAs including tRNAs and 5S ribosomal RNA, and functions as a DNA sensor in innate immune responses against viruses and intracellular bacteria. Autosomal dominant mutations cause immunodeficiency with specific susceptibility to varicella zoster virus. The gene shows very low constraint against loss-of-function variants (pLI near 0), suggesting tolerance to haploinsufficiency.

Summary from RefSeq, OMIM, UniProt
Research Assistant →

Primary Disease Associations & Inheritance

?Immunodeficiency 101 (varicella zoster virus-specific)MIM #619872
AD
0
Active trials
2
Pubs (1 yr)
24
P/LP submissions
0%
P/LP missense
1.07
LOEUF
Mechanism
Clinical SummaryPOLR3F
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
24 unique Pathogenic / Likely Pathogenic· 111 VUS of 214 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.07LOEUF
pLI 0.000
Z-score 1.35
OE 0.66 (0.421.07)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
1.65Z-score
OE missense 0.64 (0.550.76)
109 obs / 169.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.66 (0.421.07)
00.351.4
Missense OE0.64 (0.550.76)
00.61.4
Synonymous OE0.82
01.21.6
LoF obs/exp: 12 / 18.2Missense obs/exp: 109 / 169.4Syn Z: 1.11

ClinVar Variant Classifications

214 submitted variants in ClinVar

Classification Summary

Pathogenic24
VUS111
Likely Benign51
Benign18
Conflicting2
24
Pathogenic
111
VUS
51
Likely Benign
18
Benign
2
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
24
0
24
Likely Pathogenic
0
0
0
0
0
VUS
8
70
25
8
111
Likely Benign
1
0
31
19
51
Benign
0
1
13
4
18
Conflicting
2
Total9719331206

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

POLR3F · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
[Immunopatological and genetic aspects of pathogenesis of CNS lesions in VZV infection].
Skripchenko EY et al.·Zh Nevrol Psikhiatr Im S S Korsakova
2022Review
Top 2 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC