GOT2

Chr 16AR

glutamic-oxaloacetic transaminase 2

Also known as: DEE82, KAT4, KATIV, KYAT4, mitAAT

Glutamic-oxaloacetic transaminase 2 (GOT2) is a mitochondrial enzyme that catalyzes amino acid transamination reactions, plays a key role in the malate-aspartate shuttle for intracellular NAD(H) redox balance, and facilitates cellular uptake of long-chain fatty acids. Biallelic mutations cause developmental and epileptic encephalopathy 82, inherited in an autosomal recessive pattern. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.623), affecting both neurological development and seizure control.

OMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismARLOEUF 0.621 OMIM phenotype
Clinical SummaryGOT2
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.33) despite low pLI — interpret in context.
💊
Clinical Trials
12 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.62LOEUF
pLI 0.014
Z-score 2.84
OE 0.33 (0.190.62)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.39Z-score
OE missense 0.75 (0.670.85)
190 obs / 251.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.33 (0.190.62)
00.351.4
Missense OE0.75 (0.670.85)
00.61.4
Synonymous OE0.74
01.21.6
LoF obs/exp: 7 / 21.1Missense obs/exp: 190 / 251.9Syn Z: 1.93
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongGOT2-related malate-aspartate shuttle-related encephalopathyOTHERAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.74top 25%
GOF
0.4875th %ile
LOF
0.3162th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

GOT2 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

Malnutrition PregnancyMalnutrition in ChildrenMalnutrition (Calorie)

Early Life Malnutrition, Environmental Enteric Dysfunction and Microbiome Trajectories

RECRUITING
NCT07195006University of ZimbabweStarted 2025-01-27
Malnutrition in pregnancy as exposurePoor WASH living conditions as exposure
Relapsing Remitting Multiple Sclerosis (RRMS)

Indole-3-PROpionic Acid Clinical Trials - Multiple Sclerosis

RECRUITING
NCT07318129Phase NAGlostrup University Hospital, CopenhagenStarted 2026-01-26
PlaceboIndole-3-propionic acid (IPA)
Alcohol Use DisorderRisk Behavior, HealthDysbiosis

Alcohol Misuse, Gut Microbial Dysbiosis and PrEP Care Continuum: Application and Efficacy of SBIRT Intervention

RECRUITING
NCT06005298Phase NAShirish S BarveStarted 2023-08-01
Screening, Brief Intervention, Referral to Treatment (SBIRT)
Weight GainAnemia, Iron DeficiencyBirth Weight

Egg Intervention During Pregnancy in Indonesia

ACTIVE NOT RECRUITING
NCT04694235Phase NASEAMEO Regional Centre for Food and NutritionStarted 2021-02-12
Egg intervention
Alzheimer Disease (AD)

Tributyrin Treatment in Mild Alzheimer Disease: Assessment of Butyrate Effects Via the Gut-Brain

NOT YET RECRUITING
NCT06797817Phase PHASE3Universidad de AlmeriaStarted 2026-06-01
tributyrinPlacebo
Critical IllnessGastro Intestinal SurgeryEnteral Feeding Intolerance

Undiluted and Diluted Nutrition

NOT YET RECRUITING
NCT07352150Phase NAJohn Paul II Catholic University of LublinStarted 2026-02-02
Enteral fluidIntravenous fluid
Obese Patients (BMI ≥ 30 kg/m²)AnxietyDepressive Disorder

Therapeutic Potential of a Synbiotic to Improve Mental Health in Subjects With Obesity.

RECRUITING
NCT06901739Phase NACelia BañulsStarted 2025-04-15
SynbioticPlacebo
Congenital Heart Disease (CHD)

Butyric Acid Supplementation for Gut Improvement After Cardiac Surgery in Kids

NOT YET RECRUITING
NCT06882772Phase PHASE1, PHASE2University of NebraskaStarted 2026-03
2 mL SunButyrate-TGPlacebo4 mL SunButyrate-TG
Critical IllnessSurgeryEnteral Feeding Intolerance

Diluted and Undiluted Enteral Nutrition

NOT YET RECRUITING
NCT06516835Phase NAMedical University of LublinStarted 2025-01-02
Enteral fluidIntravenous fluid
Heart FailureDilated Cardiomyopathy

Myocardial Telomere Recapping Study for Dilated Cardiomyopathy

ACTIVE NOT RECRUITING
NCT05837143Phase EARLY_PHASE1Shanghai East HospitalStarted 2023-03-30
JV001
Colo-rectal Cancer

Effect of Synbiotic Supplementation on the Prevention of Mucositis in Cancer Patients Undergoing Chemotherapy

RECRUITING
NCT06576986Phase NAFederal University of Minas GeraisStarted 2023-12-12
SymbioticMaltodextrin
SymbioticUremic ToxinsGut -Microbiota

Modulation of Gut Microbiota Composition and Gut Permeability Profiles by Multispecies Synbiotic Supplementation in Hemodialysis Patients

NOT YET RECRUITING
NCT07461233Phase NATungs' Taichung Metroharbour HospitalStarted 2026-04-01
Multispecies Synbiotic Supplementation
Clinical Literature
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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
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