OAT

Chr 10AR

ornithine aminotransferase

Also known as: GACR, HOGA, OATASE, OKT

This gene encodes the mitochondrial enzyme ornithine aminotransferase, which is a key enzyme in the pathway that converts arginine and ornithine into the major excitatory and inhibitory neurotransmitters glutamate and GABA. Mutations that result in a deficiency of this enzyme cause the autosomal recessive eye disease Gyrate Atrophy. Alternatively spliced transcript variants encoding different isoforms have been described. Related pseudogenes have been defined on the X chromosome. [provided by RefSeq, Jan 2010]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Gyrate atrophy of choroid and retina with or without ornithinemiaMIM #258870
AR

Clinical highlights

Gene-disease validity (ClinGen)
ornithine aminotransferase deficiency · ARDefinitivesufficient evidence for diagnostic panels
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype), though the gene is not strongly LoF-constrained in the population — weigh truncating variants against that tolerance.Curated gene-level mechanism — a prior for triage, not a per-variant call.
4
Active trials
Pubs (1 yr)
P/LP submissions
P/LP missense
0.96
LOEUF
LOF
Mechanism· G2P

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.96LOEUF
pLI 0.000
Z-score 1.70
OE 0.61 (0.390.96)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.88Z-score
OE missense 0.84 (0.750.94)
204 obs / 242.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.61 (0.390.96)
00.351.4
Missense OE?0.84 (0.750.94)
00.61.4
Synonymous OE?0.87
01.21.6
LoF obs/exp: 13 / 21.5Missense obs/exp: 204 / 242.4Syn Z: 1.01

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

OAT · protein map & ClinVar variants

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