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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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TOR2A
torsin family 2 member A
Chromosome 9 · 9q34.11
NCBI Gene: 27433Ensembl: ENSG00000160404.19HGNC: HGNC:11996UniProt: Q5JU69
66PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
identical protein bindingendoplasmic reticulum lumennuclear envelopeATP bindingprostate carcinomamathematical abilitypreeclampsiametabolic syndrome
✦AI Summary

TOR2A (torsin family 2 member A) is a multifunctional gene located on chromosome 9 that encodes proteins involved in nuclear envelope homeostasis and bioactive peptide generation. Primary function: TOR2A encodes a torsin ATPase with redundant roles in maintaining nuclear envelope (NE) integrity, particularly in regulating nuclear pore complex (NPC) biogenesis 1. The protein localizes to the endoplasmic reticulum lumen and nuclear envelope, where it functions alongside LAP1 and LULL1 cofactors 1. Mechanism: TOR2A undergoes alternative splicing to generate preprosalusin, which is processed into salusin-alpha and salusin-beta peptides 2. These salusins increase intracellular calcium concentrations, induce cell mitogenesis, and produce hypotensive effects 2. Disease relevance: TOR2A variants have been investigated in dystonia pathogenesis, particularly blepharospasm (BSP), though highly deleterious variants are rare in BSP patients 345. Genome-wide association studies identified TOR2A as a locus associated with estimated glomerular filtration rate in sickle cell nephropathy 6. TOR2A was also identified in a prognostic signature for gastric cancer survival 7. Clinical significance: While TOR2A's role in NE homeostasis is established, its direct pathogenic contribution to dystonia remains indeterminate, requiring larger cohorts and functional validation.

Sources cited
1
TOR2A is a torsin ATPase functioning at the nuclear envelope in NPC biogenesis/homeostasis; deletion increases nuclear envelope blebbing
PMID: 27798237
2
TOR2A encodes preprosalusin, which generates salusin-alpha and salusin-beta peptides that increase intracellular Ca2+, induce mitogenesis, and cause hypotension
PMID: 12910263
3
Highly deleterious TOR2A variants are rare in blepharospasm patients; role in BSP remains indeterminate
PMID: 38076033
4
TOR2A variants identified in genetic screening of Meige syndrome and blepharospasm patients; some predicted as deleterious
PMID: 35044558
5
TOR2A variants detected in blepharospasm patients appear benign; larger cohorts and functional studies needed
PMID: 30956059
6
TOR2A identified as a genetic locus associated with eGFR in sickle cell nephropathy through GWAS
PMID: 38791464
7
TOR2A included in a 9-gene prognostic signature for gastric cancer overall survival
PMID: 35421138
Disease Associationsⓘ20
prostate carcinomaOpen Targets
0.05Suggestive
mathematical abilityOpen Targets
0.05Suggestive
preeclampsiaOpen Targets
0.03Suggestive
metabolic syndromeOpen Targets
0.01Suggestive
neuroblastomaOpen Targets
0.01Suggestive
DystoniaOpen Targets
0.01Suggestive
pulmonary hypertensionOpen Targets
0.01Suggestive
gestational diabetesOpen Targets
0.01Suggestive
brucellosisOpen Targets
0.01Suggestive
atherosclerosisOpen Targets
0.01Suggestive
benign essential blepharospasmOpen Targets
0.01Suggestive
Blepharospasm - oromandibular dystoniaOpen Targets
0.00Suggestive
blepharospasm-oromandibular dystonia syndromeOpen Targets
0.00Suggestive
pulmonary arterial hypertensionOpen Targets
0.00Suggestive
cancerOpen Targets
0.00Suggestive
cardiovascular diseaseOpen Targets
0.00Suggestive
cardiac hypertrophyOpen Targets
0.00Suggestive
hepatocellular carcinomaOpen Targets
0.00Suggestive
relapsing-remitting multiple sclerosisOpen Targets
0.00Suggestive
Torsion dystoniaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PAMProtein interaction83%REEP4Co-mentioned in literature20%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
39%
Lung
30%
Ovary
28%
Brain
13%
Heart
11%
Gene Interaction Network
Click a node to explore
TOR2APAMREEP4
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q5JU69
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.52LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.08 [0.79–1.52]
RankingsWhere TOR2A stands among ~20K protein-coding genes
  • #7,133of 20,598
    Most Researched66
  • #15,304of 17,882
    Most Constrained (LOEUF)1.52
Genes detectedTOR2A
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
PMID: 38076033
Tremor Other Hyperkinet Mov (N Y) · 2023
1.00
2
Genetic screening in patients of Meige syndrome and blepharospasm.
PMID: 35044558
Neurol Sci · 2022
0.90
3
Blepharospasm: A genetic screening study in 132 patients.
PMID: 30956059
Parkinsonism Relat Disord · 2019
0.80
4
Dissecting Torsin/cofactor function at the nuclear envelope: a genetic study.
PMID: 27798237
Mol Biol Cell · 2016
0.70
5
[Salusin].
PMID: 16895179
Nihon Rinsho · 2006
0.60