CBLN2

Chr 18

cerebellin 2 precursor

The CBLN2 protein acts as a synaptic organizer that is essential for long-term maintenance of excitatory synapses, functioning in trans-synaptic complexes with neurexins and GRID1 to regulate NMDA and AMPA receptor activity at hippocampal synapses. Mutations in CBLN2 cause neurodevelopmental disorders with intellectual disability and seizures, inherited in an autosomal recessive pattern. The gene shows relatively low constraint to loss-of-function variation (pLI 0.12, LOEUF 1.17).

Summary from RefSeq, UniProt
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0
Active trials
10
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.17
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryCBLN2
Population Constraint (gnomAD)
Low constraint (pLI 0.12) — 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
1.17LOEUF
pLI 0.122
Z-score 1.35
OE 0.37 (0.151.17)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
1.87Z-score
OE missense 0.52 (0.420.64)
62 obs / 119.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.37 (0.151.17)
00.351.4
Missense OE0.52 (0.420.64)
00.61.4
Synonymous OE1.13
01.21.6
LoF obs/exp: 2 / 5.4Missense obs/exp: 62 / 119.4Syn Z: -0.78
DN
0.74top 25%
GOF
0.74top 25%
LOF
0.3648th %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

CBLN2 · 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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