GTF3C5

Chr 9

general transcription factor IIIC subunit 5

Also known as: TFIIIC63, TFIIICepsilon, TFiiiC2-63

The GTF3C5 protein functions as an integral component of the TFIIIC2 transcription factor complex that directly binds tRNA and viral RNA promoters to enable RNA polymerase III-mediated transcription of tRNA and 5S ribosomal RNA. The extremely low pLI score and LOEUF score of 0.89 suggest the gene is relatively tolerant to loss-of-function variants, and no specific disease associations are established in the provided data. Clinical significance of GTF3C5 variants in pediatric neurogenetic disorders remains to be determined.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.89
Clinical SummaryGTF3C5
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
36 unique Pathogenic / Likely Pathogenic· 109 VUS of 166 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.89LOEUF
pLI 0.000
Z-score 2.02
OE 0.59 (0.410.89)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.77Z-score
OE missense 0.88 (0.800.97)
292 obs / 331.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.59 (0.410.89)
00.351.4
Missense OE0.88 (0.800.97)
00.61.4
Synonymous OE1.11
01.21.6
LoF obs/exp: 17 / 28.7Missense obs/exp: 292 / 331.5Syn Z: -1.00

ClinVar Variant Classifications

166 submitted variants in ClinVar

Classification Summary

Pathogenic35
Likely Pathogenic1
VUS109
Likely Benign9
35
Pathogenic
1
Likely Pathogenic
109
VUS
9
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
35
0
35
Likely Pathogenic
0
0
1
0
1
VUS
1
92
16
0
109
Likely Benign
0
4
1
4
9
Benign
0
0
0
0
0
Total196534154

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

View in ClinVar →

Protein Context — Lollipop Plot

GTF3C5 · 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
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found