TOR1AIP2

Chr 1

torsin 1A interacting protein 2

Also known as: IFRG15, LULL1, NET9

One of the two protein isoforms encoded by this gene is a type II integral membrane protein found in the endoplasmic reticulum (ER). The encoded protein is a cofactor for the ATPase TorsinA, regulating the amount of TorsinA present in the ER compared to that found in the nuclear envelope. Defects in this protein are a cause of early onset primary dystonia, a neuromuscular disease. The other isoform encoded by this gene is an interferon alpha responsive protein whose cellular role has yet to be determined. [provided by RefSeq, Mar 2017]

OMIMResearchGenerating clinical summary…
LOEUF 1.40
Clinical SummaryTOR1AIP2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
1 unique Pathogenic / Likely Pathogenic· 2 VUS of 20 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.40LOEUF
pLI 0.000
Z-score 0.39
OE 0.89 (0.591.40)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.80Z-score
OE missense 0.86 (0.760.96)
210 obs / 245.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.89 (0.591.40)
00.351.4
Missense OE?0.86 (0.760.96)
00.61.4
Synonymous OE?1.02
01.21.6
LoF obs/exp: 14 / 15.7Missense obs/exp: 210 / 245.3Syn Z: -0.17

ClinVar Variant Classifications

20 submitted variants in ClinVar

Classification Summary

Pathogenic1
VUS2
Likely Benign1
1
Pathogenic
2
VUS
1
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
1
0
0
0
1
Likely Pathogenic
0
0
0
0
0
VUS
0
2
0
0
2
Likely Benign
0
1
0
0
1
Benign
0
0
0
0
0
Total13004

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

View in ClinVar →

30 pathogenic / likely-pathogenic (of 33) ClinVar copy-number / structural variants overlap TOR1AIP2 — these span large chromosomal regions, not the gene specifically, and are excluded from the counts above. Explore in CNV tools →

Protein Context — Lollipop Plot

TOR1AIP2 · 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.

Search ClinicalTrials.gov →