FAM118B

Chr 11

SIR2 antiphage like 1

Also known as: FAM118B, SIRal

The FAM118B protein enables identical protein binding and may contribute to Cajal body formation, which are nuclear structures involved in RNA processing. Mutations cause autosomal recessive developmental and epileptic encephalopathy with microcephaly. This gene shows moderate constraint against loss-of-function variants.

Summary from RefSeq, UniProt
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0
Active trials
3
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.68
LOEUF
GOF
Mechanism· predicted
Clinical SummaryFAM118B
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.34) 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
0.68LOEUF
pLI 0.019
Z-score 2.55
OE 0.34 (0.190.68)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.96Z-score
OE missense 0.80 (0.700.92)
154 obs / 191.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.34 (0.190.68)
00.351.4
Missense OE0.80 (0.700.92)
00.61.4
Synonymous OE0.84
01.21.6
LoF obs/exp: 6 / 17.5Missense obs/exp: 154 / 191.6Syn Z: 1.10
DN
0.5967th %ile
GOF
0.6930th %ile
LOF
0.2289th %ile

The highest-scoring mechanism for this gene is gain-of-function.

GOFprediction 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

FAM118B · 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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Clinical Literature
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