TRIP11

Chr 14

thyroid hormone receptor interactor 11

Also known as: ACG1A, CEV14, GMAP-210, GMAP210, ODCD, ODCD1, TRIP-11, TRIP230

The protein functions as a membrane tether essential for maintaining Golgi structure and efficient trafficking in the early secretory pathway between the endoplasmic reticulum and Golgi complex. Mutations cause achondrogenesis type IA and odontochondrodysplasia 1, severe skeletal disorders that primarily affect cartilage and bone development. The gene follows autosomal recessive inheritance and is highly constrained against loss-of-function variants.

ResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismLOEUF 0.50
Clinical SummaryTRIP11
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Gene-Disease Validity (ClinGen)
TRIP11-related skeletal dysplasia · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.50LOEUF
pLI 0.000
Z-score 5.72
OE 0.39 (0.300.50)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.52Z-score
OE missense 0.95 (0.901.01)
903 obs / 947.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.39 (0.300.50)
00.351.4
Missense OE0.95 (0.901.01)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 39 / 101.1Missense obs/exp: 903 / 947.7Syn Z: -0.89
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveTRIP11-related achondrogenesisLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.7033th %ile
GOF
0.4283th %ile
LOF
0.3162th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TRIP11 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

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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