TCIRG1

Chr 11

T cell immune regulator 1, ATPase H+ transporting V0 subunit a3

Also known as: ATP6N1C, ATP6V0A3, Atp6i, OC-116kDa, OC116, OPTB1, Stv1, TIRC7

This gene encodes a subunit of a large protein complex known as a vacuolar H+-ATPase (V-ATPase). The protein complex acts as a pump to move protons across the membrane. This movement of protons helps regulate the pH of cells and their surrounding environment. V-ATPase dependent organelle acidification is necessary for such intracellular processes as protein sorting, zymogen activation, and receptor-mediated endocytosis. V-ATPase is comprised of a cytosolic V1 domain and a transmembrane V0 domain. Alternative splicing results in multiple transcript variants. Mutations in this gene are associated with infantile malignant osteopetrosis. [provided by RefSeq, May 2017]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtOsteopetrosis, autosomal recessive 1

Clinical highlights

Gene-disease validity (ClinGen)
autosomal recessive osteopetrosis 1 · ARDefinitivesufficient evidence for diagnostic panels2 gene-disease associations curated in total
1
Active trials
35
Pubs (1 yr)
P/LP submissions
P/LP missense
0.87
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — TCIRG1
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.87LOEUF
pLI 0.000
Z-score 2.26
OE 0.63 (0.460.87)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.63Z-score
OE missense 0.92 (0.860.99)
489 obs / 529.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.63 (0.460.87)
00.351.4
Missense OE?0.92 (0.860.99)
00.61.4
Synonymous OE?1.04
01.21.6
LoF obs/exp: 27 / 43.0Missense obs/exp: 489 / 529.6Syn Z: -0.47

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TCIRG1 · protein map & ClinVar variants

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