GPT2

Chr 16AR

glutamic--pyruvic transaminase 2

Also known as: ALT2, GPT 2, MRT49, NEDSPM

This gene encodes a mitochondrial alanine transaminase, a pyridoxal enzyme that catalyzes the reversible transamination between alanine and 2-oxoglutarate to generate pyruvate and glutamate. Alanine transaminases play roles in gluconeogenesis and amino acid metabolism in many tissues including skeletal muscle, kidney, and liver. Activating transcription factor 4 upregulates this gene under metabolic stress conditions in hepatocyte cell lines. A loss of function mutation in this gene has been associated with developmental encephalopathy. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2015]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Neurodevelopmental disorder with microcephaly and spastic paraplegiaMIM #616281
AR

Clinical highlights

Gene-disease validity (ClinGen)
glutamate pyruvate transaminase 2 deficiency · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
43
Pubs (1 yr)
P/LP submissions
P/LP missense
0.70
LOEUF
DN
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.70LOEUF
pLI 0.000
Z-score 2.69
OE 0.41 (0.250.70)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
1.88Z-score
OE missense 0.70 (0.630.78)
216 obs / 308.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.41 (0.250.70)
00.351.4
Missense OE?0.70 (0.630.78)
00.61.4
Synonymous OE?0.94
01.21.6
LoF obs/exp: 10 / 24.3Missense obs/exp: 216 / 308.8Syn Z: 0.57

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

GPT2 · 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 →