TOR2A

Chr 9

torsin family 2 member A

Also known as: TORP1

This gene encodes an AAA family ATPase with similarity to Clp proteases and heat shock proteins, and produces salusin peptides that regulate blood pressure and have antimicrobial properties. Mutations cause autosomal recessive dystonia-26 with infantile onset, characterized by early-onset generalized dystonia and developmental delays. The gene shows moderate tolerance to loss-of-function variants based on population genetics data.

OMIMResearchSummary from RefSeq
DNmechanismLOEUF 0.78
Clinical SummaryTOR2A
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.30) 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
0.78LOEUF
pLI 0.136
Z-score 2.03
OE 0.30 (0.140.78)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.25Z-score
OE missense 0.95 (0.841.07)
181 obs / 190.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.30 (0.140.78)
00.351.4
Missense OE0.95 (0.841.07)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 3 / 9.9Missense obs/exp: 181 / 190.6Syn Z: 0.17
DN
0.76top 25%
GOF
0.6248th %ile
LOF
0.2777th %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

TOR2A · 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 →