CSMD3

Chr 8

CUB and Sushi multiple domains 3

CSMD3 encodes a protein involved in dendrite development and is predicted to localize to the plasma membrane. The gene is highly constrained against loss-of-function variation (LOEUF = 0.30), suggesting intolerance to protein-truncating variants. Mutations in CSMD3 have been associated with neurodevelopmental disorders, though the clinical phenotype and inheritance pattern require further characterization.

Summary from RefSeq, UniProt
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0
Active trials
17
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.30
LOEUF· LoF intol.
Multiple*
Mechanism· predicted
Clinical SummaryCSMD3
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.23) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.30LOEUF
pLI 0.057
Z-score 9.96
OE 0.23 (0.180.30)
Highly constrained

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

Missense Constraint
1.14Z-score
OE missense 0.93 (0.890.96)
1813 obs / 1954.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.23 (0.180.30)
00.351.4
Missense OE0.93 (0.890.96)
00.61.4
Synonymous OE1.13
01.21.6
LoF obs/exp: 46 / 196.9Missense obs/exp: 1813 / 1954.8Syn Z: -2.61
DN
0.6841th %ile
GOF
0.6930th %ile
LOF
0.3647th %ile

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

GOFprediction above median
DNprediction 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

CSMD3 · 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.

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Clinical Literature
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