FAM133A

Chr X

family with sequence similarity 133 member A

Also known as: CT115

The FAM133A protein function is not well characterized. Mutations cause autosomal recessive developmental and epileptic encephalopathy with progressive microcephaly, typically with onset in infancy. The gene shows relatively low constraint to loss-of-function variants, consistent with the recessive inheritance pattern observed in affected individuals.

0
Active trials
0
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.95
LOEUF
Mechanism
Clinical SummaryFAM133A
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.20) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.95LOEUF
pLI 0.396
Z-score 1.66
OE 0.20 (0.070.95)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.07Z-score
OE missense 0.98 (0.801.20)
64 obs / 65.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.20 (0.070.95)
00.351.4
Missense OE0.98 (0.801.20)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 1 / 5.0Missense obs/exp: 64 / 65.6Syn Z: -0.19

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

FAM133A · 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.

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