FAM13A

Chr 4

family with sequence similarity 13 member A

Also known as: ARHGAP48, FAM13A1

The FAM13A protein functions as a GTPase activator that regulates small GTPase-mediated signal transduction in the cytosol. This gene is extremely intolerant to loss-of-function mutations and has been implicated in chronic obstructive pulmonary disease, though specific pediatric neurogenetic disorders caused by FAM13A mutations are not established in the provided data. The inheritance pattern for FAM13A-related conditions has not been determined from the available information.

Summary from RefSeq
Research Assistant →
0
Active trials
17
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.83
LOEUF
DN
Mechanism· predicted
Clinical SummaryFAM13A
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
0.83LOEUF
pLI 0.000
Z-score 2.64
OE 0.63 (0.490.83)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.80Z-score
OE missense 0.91 (0.840.97)
510 obs / 563.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.63 (0.490.83)
00.351.4
Missense OE0.91 (0.840.97)
00.61.4
Synonymous OE0.90
01.21.6
LoF obs/exp: 38 / 60.1Missense obs/exp: 510 / 563.5Syn Z: 1.13
DN
0.7230th %ile
GOF
0.5660th %ile
LOF
0.3452th %ile

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

FAM13A · 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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