TAPT1

Chr 4AR

transmembrane anterior posterior transformation 1

Also known as: CMVFR, OCLSBG

The protein localizes to the centrosome and ciliary basal body and is essential for primary cilium formation and normal cartilage and bone development. Mutations cause osteochondrodysplasia, complex lethal, Symoens-Barnes-Gistelinck type, a severe congenital skeletal disorder with undermineralization of the intrauterine skeleton. This condition follows autosomal recessive inheritance.

Summary from RefSeq, OMIM, UniProt
Research Assistant →

Primary Disease Associations & Inheritance

Osteochondrodysplasia, complex lethal, Symoens-Barnes-Gistelinck typeMIM #616897
AR
0
Active trials
3
Pubs (1 yr)
61
P/LP submissions
2%
P/LP missense
0.56
LOEUF
LOF
Mechanism· G2P
Clinical SummaryTAPT1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.30) despite low pLI — interpret in context.
📋
ClinVar Variants
60 unique Pathogenic / Likely Pathogenic· 105 VUS of 403 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.56LOEUF
pLI 0.032
Z-score 3.13
OE 0.30 (0.170.56)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.92Z-score
OE missense 0.65 (0.570.74)
157 obs / 241.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.30 (0.170.56)
00.351.4
Missense OE0.65 (0.570.74)
00.61.4
Synonymous OE0.92
01.21.6
LoF obs/exp: 7 / 23.3Missense obs/exp: 157 / 241.0Syn Z: 0.59
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedTAPT1-related cataractOTHERAR
strongTAPT1-related complex lethal osteochondrodysplasiaLOFAR

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

DN
0.7132th %ile
GOF
0.6442th %ile
LOF
0.3647th %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

403 submitted variants in ClinVar

Classification Summary

Pathogenic52
Likely Pathogenic8
VUS105
Likely Benign151
Benign72
Conflicting4
52
Pathogenic
8
Likely Pathogenic
105
VUS
151
Likely Benign
72
Benign
4
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
3
1
48
0
52
Likely Pathogenic
3
0
5
0
8
VUS
0
93
11
1
105
Likely Benign
0
5
81
65
151
Benign
0
1
67
4
72
Conflicting
4
Total610021270392

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

View in ClinVar →

Protein Context — Lollipop Plot

TAPT1 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC