PABPC3

Chr 13

poly(A) binding protein cytoplasmic 3

Also known as: PABP3, PABPL3, tPABP

PABPC3 encodes a poly(A)-binding protein that binds to the poly(A) tail of mRNA and regulates cytoplasmic mRNA metabolism and translation. The gene is extremely intolerant to loss-of-function variants, indicating it is likely essential for normal cellular function. However, no specific disease phenotypes have been definitively associated with PABPC3 mutations in the current literature.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 1.67
Clinical SummaryPABPC3
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
33 unique Pathogenic / Likely Pathogenic· 4 VUS of 38 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.67LOEUF
pLI 0.000
Z-score -0.61
OE 1.16 (0.811.67)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.78Z-score
OE missense 1.11 (1.031.21)
410 obs / 368.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.16 (0.811.67)
00.351.4
Missense OE1.11 (1.031.21)
00.61.4
Synonymous OE0.92
01.21.6
LoF obs/exp: 20 / 17.3Missense obs/exp: 410 / 368.1Syn Z: 0.69
DN
0.7325th %ile
GOF
0.4184th %ile
LOF
0.4136th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

38 submitted variants in ClinVar

Classification Summary

Pathogenic31
Likely Pathogenic2
VUS4
Likely Benign1
31
Pathogenic
2
Likely Pathogenic
4
VUS
1
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
31
Likely Pathogenic
2
VUS
4
Likely Benign
1
Benign
0
Total38

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

PABPC3 · 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.

Search ClinicalTrials.gov →
Clinical Literature
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