TPRX1

Chr 19

tetrapeptide repeat homeobox 1

Also known as: TPRX

Homeobox genes encode DNA-binding proteins, many of which are thought to be involved in early embryonic development. Homeobox genes encode a DNA-binding domain of 60 to 63 amino acids referred to as the homeodomain. This gene is a member of the TPRX homeobox gene family. [provided by RefSeq, Jul 2008]

OMIMResearchGenerating clinical summary…
DNmechanismLOEUF 1.79
Clinical SummaryTPRX1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.00) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.79LOEUF
pLI 0.359
Z-score 0.78
OE 0.00 (0.001.79)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.34Z-score
OE missense 1.06 (0.961.19)
236 obs / 221.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.00 (0.001.79)
00.351.4
Missense OE?1.06 (0.961.19)
00.61.4
Synonymous OE?1.09
01.21.6
LoF obs/exp: 0 / 0.7Missense obs/exp: 236 / 221.9Syn Z: -0.68

This gene — mechanism propensity

DN
0.7132th %ile
GOF
0.6248th %ile
LOF
0.4825th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction 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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TPRX1 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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