CACNG2

Chr 22AD

calcium voltage-gated channel auxiliary subunit gamma 2

Also known as: MRD10

CACNG2 encodes a transmembrane AMPA receptor regulatory protein (TARP) that controls trafficking and gating properties of AMPA-selective glutamate receptors, which mediate fast excitatory neurotransmission and are crucial for synaptic plasticity, learning and memory. Mutations cause autosomal dominant intellectual developmental disorder. This gene is highly constrained against loss-of-function variants (pLI 0.92, LOEUF 0.38), suggesting intolerance to protein disruption.

OMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismADLOEUF 0.381 OMIM phenotype
Clinical SummaryCACNG2
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Gene-Disease Validity (ClinGen)
complex neurodevelopmental disorder · ADLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.92). One damaged copy is likely sufficient to cause disease.
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ClinVar Variants
17 unique Pathogenic / Likely Pathogenic· 64 VUS of 97 total submissions
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.38LOEUF
pLI 0.923
Z-score 3.03
OE 0.08 (0.030.38)
Highly constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
2.09Z-score
OE missense 0.59 (0.510.68)
119 obs / 202.9 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.08 (0.030.38)
00.351.4
Missense OE0.59 (0.510.68)
00.61.4
Synonymous OE1.01
01.21.6
LoF obs/exp: 1 / 12.6Missense obs/exp: 119 / 202.9Syn Z: -0.07
DN
0.5771th %ile
GOF
0.6345th %ile
LOF
0.65top 25%

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

LOFprediction above median · LOEUF 0.38
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. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

97 submitted variants in ClinVar

Classification Summary

Pathogenic16
Likely Pathogenic1
VUS64
Likely Benign11
Benign1
16
Pathogenic
1
Likely Pathogenic
64
VUS
11
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
16
0
16
Likely Pathogenic
0
0
1
0
1
VUS
3
49
11
1
64
Likely Benign
0
1
3
7
11
Benign
0
0
0
1
1
Total35031993

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

CACNG2 · 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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