PCF11

Chr 11

PCF11 cleavage and polyadenylation factor subunit

PCF11 encodes a component of the pre-mRNA cleavage complex II that promotes transcription termination by RNA polymerase II. Mutations cause autosomal dominant neurodevelopmental disorders with intellectual disability and developmental delay. This gene is extremely intolerant to loss-of-function variants, indicating that even small changes can have significant clinical consequences.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanism
Clinical SummaryPCF11
📋
ClinVar Variants
15 unique Pathogenic / Likely Pathogenic· 164 VUS of 208 total submissions
Some data sources returned errors (1)

gnomad: TimeoutError: The operation was aborted due to timeout

Population Genetics & Constraint

Constraint data not available from gnomAD.

DN
0.17100th %ile
GOF
0.09100th %ile
LOF
0.86top 5%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median

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

ClinVar Variant Classifications

208 submitted variants in ClinVar

Classification Summary

Pathogenic15
VUS164
Likely Benign5
Benign4
15
Pathogenic
164
VUS
5
Likely Benign
4
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
15
0
15
Likely Pathogenic
0
0
0
0
0
VUS
1
157
6
0
164
Likely Benign
0
3
0
2
5
Benign
0
2
0
2
4
Total1162214188

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

View in ClinVar →

Protein Context — Lollipop Plot

PCF11 · 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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