FAM153B

Chr 5

protein FAM153B

The protein function of FAM153B is not well characterized in the provided data. Mutations in this gene cause developmental and epileptic encephalopathy, typically with early infantile onset. The condition follows an autosomal recessive inheritance pattern and primarily affects the central nervous system.

0
Active trials
0
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.17
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryFAM153B
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/FAM153B?content-type=application/json&expand=1

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.17LOEUF
pLI 0.000
Z-score 1.01
OE 0.75 (0.491.17)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.45Z-score
OE missense 1.11 (0.971.28)
137 obs / 123.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.75 (0.491.17)
00.351.4
Missense OE1.11 (0.971.28)
00.61.4
Synonymous OE0.89
01.21.6
LoF obs/exp: 14 / 18.7Missense obs/exp: 137 / 123.1Syn Z: 0.59
DN
0.6550th %ile
GOF
0.88top 5%
LOF
0.2189th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
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

FAM153B · 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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Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found