TRIM63

Chr 1AR

tripartite motif containing 63

Also known as: CMH31, IRF, MURF1, MURF2, RNF28, SMRZ

The protein is an E3 ubiquitin ligase that localizes to myofibrils and mediates the ubiquitination and degradation of muscle proteins including myosin heavy chain, creatine kinase, and cardiac troponin I, playing a crucial role in muscle protein turnover and atrophy. Mutations cause hypertrophic cardiomyopathy with autosomal dominant inheritance. The gene shows very low constraint against loss-of-function variants, suggesting tolerance to such mutations.

GeneReviewsOMIMResearchSummary from RefSeq, UniProt
MultiplemechanismARLOEUF 1.471 OMIM phenotype
Clinical SummaryTRIM63
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Gene-Disease Validity (ClinGen)
hypertrophic cardiomyopathy · ADDisputed

Disputed — evidence questions this relationship

2 total gene-disease associations curated

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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ClinVar Variants
12 unique Pathogenic / Likely Pathogenic· 97 VUS of 171 total submissions
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
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GeneReview available — TRIM63
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
1.47LOEUF
pLI 0.000
Z-score -0.01
OE 1.00 (0.701.47)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.18Z-score
OE missense 1.03 (0.921.16)
212 obs / 204.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.00 (0.701.47)
00.351.4
Missense OE1.03 (0.921.16)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 19 / 19.0Missense obs/exp: 212 / 204.9Syn Z: -0.41
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedTRIM63-related hypertrophic cardiomyopathyOTHERAD
DN
0.75top 25%
GOF
0.72top 25%
LOF
0.2970th %ile

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

DNprediction above median
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.

ClinVar Variant Classifications

171 submitted variants in ClinVar

Classification Summary

Pathogenic9
Likely Pathogenic3
VUS97
Likely Benign34
Benign21
Conflicting7
9
Pathogenic
3
Likely Pathogenic
97
VUS
34
Likely Benign
21
Benign
7
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
2
1
6
0
9
Likely Pathogenic
3
0
0
0
3
VUS
5
87
5
0
97
Likely Benign
0
4
12
18
34
Benign
0
0
18
3
21
Conflicting
7
Total10924121171

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

View in ClinVar →

Protein Context — Lollipop Plot

TRIM63 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
Open Research Assistant →