FAM177A1

Chr 14AR

family with sequence similarity 177 member A1

Also known as: C14orf24, NEDWMG

This gene encodes a member of a conserved protein family with unknown specific function. Biallelic mutations cause autosomal recessive neurodevelopmental disorder with white matter abnormalities and gait disturbance. The gene shows tolerance to loss-of-function variation in the general population (pLI 0.001, LOEUF 1.139).

OMIMResearchSummary from RefSeq, OMIM
LOFmechanismARLOEUF 1.141 OMIM phenotype
Clinical SummaryFAM177A1
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
1.14LOEUF
pLI 0.001
Z-score 1.26
OE 0.58 (0.321.14)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.30Z-score
OE missense 1.08 (0.931.25)
131 obs / 121.6 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.58 (0.321.14)
00.351.4
Missense OE1.08 (0.931.25)
00.61.4
Synonymous OE1.16
01.21.6
LoF obs/exp: 6 / 10.4Missense obs/exp: 131 / 121.6Syn Z: -0.80
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
moderateFAM177A1-related neurodevelopmental disorder with macrocephalyLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6454th %ile
GOF
0.74top 25%
LOF
0.2872th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

FAM177A1 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC