FAM171A1

Chr 10

family with sequence similarity 171 member A1

Also known as: APCN, C10orf38

FAM171A1 encodes a plasma membrane protein that regulates cytoskeletal dynamics and actin stress fiber formation. The gene is highly constrained against loss-of-function variants (pLI 0.99, LOEUF 0.30), but no confirmed disease associations have been established. Mutations in this gene have not been definitively linked to human disease phenotypes.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
1
Pubs (1 yr)
17
P/LP submissions
0%
P/LP missense
0.30
LOEUF· LoF intol.
LOF
Mechanism· predicted
Clinical SummaryFAM171A1
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.99). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
17 unique Pathogenic / Likely Pathogenic· 154 VUS of 186 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.30LOEUF
pLI 0.985
Z-score 4.68
OE 0.14 (0.070.30)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
0.44Z-score
OE missense 0.95 (0.881.02)
511 obs / 539.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.14 (0.070.30)
00.351.4
Missense OE0.95 (0.881.02)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 5 / 34.8Missense obs/exp: 511 / 539.7Syn Z: 0.10
DN
0.3594th %ile
GOF
0.4874th %ile
LOF
0.73top 10%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.30

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

186 submitted variants in ClinVar

Classification Summary

Pathogenic17
VUS154
Likely Benign3
Benign1
17
Pathogenic
154
VUS
3
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
17
0
17
Likely Pathogenic
0
0
0
0
0
VUS
0
154
0
0
154
Likely Benign
0
3
0
0
3
Benign
0
0
1
0
1
Total0157180175

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

FAM171A1 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗