SDCCAG8

Chr 1AR

SHH signaling and ciliogenesis regulator SDCCAG8

Also known as: BBS16, CCCAP, CCCAP SLSN7, HSPC085, NPHP10, NY-CO-8, SLSN7, hCCCAP

This gene encodes a centrosome-associated protein that is essential for ciliogenesis and plays a critical role in establishing cell polarity and epithelial lumen formation. Mutations cause autosomal recessive ciliopathies including Bardet-Biedl syndrome 16 and Senior-Loken syndrome 7, which affect multiple organ systems including the retina, kidneys, and other ciliated tissues. The gene is extremely intolerant to loss-of-function variants (pLI ~0), indicating that complete loss of protein function is likely pathogenic.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.782 OMIM phenotypes
Clinical SummarySDCCAG8
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Gene-Disease Validity (ClinGen)
Bardet-Biedl syndrome 16 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
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GeneReview available — SDCCAG8
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.78LOEUF
pLI 0.000
Z-score 2.71
OE 0.56 (0.410.78)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.10Z-score
OE missense 1.01 (0.931.11)
367 obs / 361.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.56 (0.410.78)
00.351.4
Missense OE1.01 (0.931.11)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 25 / 44.5Missense obs/exp: 367 / 361.7Syn Z: -0.52
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveSDCCAG8-related Senior-Loken syndromeLOFAR

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

DN
0.7035th %ile
GOF
0.6051th %ile
LOF
0.3454th %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

SDCCAG8 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold
Clinical Literature
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