CFAP157

Chr 9

cilia and flagella associated protein 157

Also known as: C9orf117

The protein is specifically required during spermatogenesis for flagellum morphogenesis and sperm motility, with predicted microtubule binding activity and involvement in sperm axoneme assembly. Mutations cause primary ciliary dyskinesia with or without situs inversus, inherited in an autosomal recessive pattern. The gene shows very low constraint against loss-of-function variants, consistent with a recessive disorder affecting primarily reproductive function.

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 1.16
Clinical SummaryCFAP157
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
1.16LOEUF
pLI 0.000
Z-score 0.95
OE 0.80 (0.561.16)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.75Z-score
OE missense 0.88 (0.800.97)
271 obs / 308.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.80 (0.561.16)
00.351.4
Missense OE0.88 (0.800.97)
00.61.4
Synonymous OE0.85
01.21.6
LoF obs/exp: 20 / 25.2Missense obs/exp: 271 / 308.1Syn Z: 1.35
DN
0.79top 25%
GOF
0.74top 25%
LOF
0.2386th %ile

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

DNprediction above median
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

CFAP157 · 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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