POU5F1B

Chr 8

POU class 5 homeobox 1B

Also known as: OCT4-PG1, OCT4PG1, OTF3C, OTF3P1, POU5F1P1, POU5F1P4, POU5FLC20, POU5FLC8

POU5F1B encodes a transcription factor with weak transcriptional activator activity that may function in eye development. Mutations cause autosomal recessive developmental and epileptic encephalopathy with microcephaly and simplified gyral pattern, typically presenting in infancy. The gene is not highly constrained against loss-of-function variants, which is consistent with its recessive inheritance pattern.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 1.85
Clinical SummaryPOU5F1B
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
1.85LOEUF
pLI 0.000
Z-score -0.19
OE 1.10 (0.561.85)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.36Z-score
OE missense 1.07 (0.961.20)
205 obs / 191.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.10 (0.561.85)
00.351.4
Missense OE1.07 (0.961.20)
00.61.4
Synonymous OE1.12
01.21.6
LoF obs/exp: 5 / 4.6Missense obs/exp: 205 / 191.2Syn Z: -0.88
DN
0.6552th %ile
GOF
0.4677th %ile
LOF
0.54top 25%

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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

POU5F1B · 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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