CFAP410

Chr 21AR

cilia and flagella associated protein 410

Also known as: C21orf2, LRRC76, RDMS, SMDAX, YF5/A2

CFAP410 encodes a protein with leucine-rich repeats that functions in cilia formation and maintenance, cytoskeletal organization, and DNA damage repair. Mutations cause autosomal recessive retinal dystrophy with macular staphyloma and spondylometaphyseal dysplasia with axial involvement, affecting both ocular and skeletal systems. The gene is not highly constrained against loss-of-function variants (LOEUF 1.396).

Summary from RefSeq, OMIM, UniProt
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Primary Disease Associations & Inheritance

Retinal dystrophy with macular staphylomaMIM #617547
AR
Spondylometaphyseal dysplasia, axialMIM #602271
AR
0
Active trials
7
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.40
LOEUF
LOF
Mechanism· G2P
Clinical SummaryCFAP410
🧬
Gene-Disease Validity (ClinGen)
amyotrophic lateral sclerosis · SDLimited

Limited evidence — not for standalone diagnostic reporting

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
1.40LOEUF
pLI 0.000
Z-score 0.52
OE 0.85 (0.531.40)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.16Z-score
OE missense 1.03 (0.931.15)
244 obs / 236.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.85 (0.531.40)
00.351.4
Missense OE1.03 (0.931.15)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 11 / 13.0Missense obs/exp: 244 / 236.9Syn Z: -0.53
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveCFAP410-related retinal dystrophy with or without axial spondylometaphyseal dysplasiaLOFAR

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

DN
0.6840th %ile
GOF
0.6638th %ile
LOF
0.3746th %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

CFAP410 · 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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