TRIP12

Chr 2AD

thyroid hormone receptor interactor 12

Also known as: MRD49, TRIP-12, TRIPC, ULF

The encoded E3 ubiquitin-protein ligase degrades the p19ARF tumor suppressor isoform and regulates USP7 stability in DNA damage response pathways. Loss-of-function mutations cause autosomal dominant intellectual developmental disorder. The gene is extremely intolerant to loss-of-function variants, indicating haploinsufficiency as the disease mechanism.

OMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismADLOEUF 0.061 OMIM phenotype
Clinical SummaryTRIP12
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Gene-Disease Validity (ClinGen)
complex neurodevelopmental disorder · ADDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
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ClinVar Variants
87 unique Pathogenic / Likely Pathogenic· 326 VUS of 567 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

Dual constrained — LoF & missense intolerant
LoF Constraint
0.06LOEUF
pLI 1.000
Z-score 9.34
OE 0.02 (0.010.06)
Highly constrained

Among the most LoF-intolerant genes (~top 3%)

Missense Constraint
4.64Z-score
OE missense 0.60 (0.560.64)
639 obs / 1065.2 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.02 (0.010.06)
00.351.4
Missense OE0.60 (0.560.64)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 2 / 105.5Missense obs/exp: 639 / 1065.2Syn Z: -0.76
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveTRIP12-related intellectual disability with or without autism spectrum disorderLOFAD
DN
0.2299th %ile
GOF
0.2597th %ile
LOF
0.84top 5%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · 1 literature citation · 56% of P/LP variants are LoF · LOEUF 0.06

Literature Evidence

LOFHaploinsufficiency of the E3 ubiquitin-protein ligase gene TRIP12 causes intellectual disability with or without autism spectrum disorders, speech delay, and dysmorphic features.PMID:28251352

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

567 submitted variants in ClinVar

Classification Summary

Pathogenic63
Likely Pathogenic24
VUS326
Likely Benign84
Benign4
Conflicting5
63
Pathogenic
24
Likely Pathogenic
326
VUS
84
Likely Benign
4
Benign
5
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
36
1
26
0
63
Likely Pathogenic
13
6
5
0
24
VUS
4
292
30
0
326
Likely Benign
0
31
12
41
84
Benign
0
0
2
2
4
Conflicting
5
Total533307543506

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

View in ClinVar →

Protein Context — Lollipop Plot

TRIP12 · 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 →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC