TAT

Chr 16

tyrosine aminotransferase

This nuclear gene encodes a mitochondrial protein tyrosine aminotransferase which is present in the liver and catalyzes the conversion of L-tyrosine into p-hydroxyphenylpyruvate. Mutations in this gene cause tyrosinemia (type II, Richner-Hanhart syndrome), a disorder accompanied by major skin and corneal lesions, with possible cognitive disability. A regulator gene for tyrosine aminotransferase is X-linked. [provided by RefSeq, Jul 2008]

ResearchGenerating clinical summary…
LOFmechanismLOEUF 0.61
Clinical SummaryTAT
🧬
Gene-Disease Validity (ClinGen)
tyrosinemia type II · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.34) despite low pLI — interpret in context.
💊
Clinical Trials
9 active or recruiting trials — potential therapeutic options may be available
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?
0.61LOEUF
pLI 0.007
Z-score 2.97
OE 0.34 (0.200.61)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.76Z-score
OE missense 0.87 (0.780.97)
220 obs / 254.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.34 (0.200.61)
00.351.4
Missense OE?0.87 (0.780.97)
00.61.4
Synonymous OE?0.95
01.21.6
LoF obs/exp: 8 / 23.5Missense obs/exp: 220 / 254.1Syn Z: 0.38
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveTAT-related tyrosinemiaLOFAR

This gene — mechanism propensity

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

DN
0.76top 25%
GOF
0.5954th %ile
LOF
0.2582th %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

TAT · 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

Severe Hemophilia A

To Evaluate the Safety and Efficacy of GS1191-0445 Injection in the Treatment of Severe Hemophilia A

ACTIVE NOT RECRUITING
NCT07548411Phase PHASE1, PHASE2Gritgen Therapeutics Co., Ltd.Started 2023-07-10
GS1191-0445 injection
Prostate Cancer

Study of Talazoparib With Enzalutamide in Men With DDR Gene Mutated mCSPC

ACTIVE NOT RECRUITING
NCT04821622Phase PHASE3PfizerStarted 2021-05-12
talazoparib plus enzalutamidePlacebo plus enzalutamide
Coronary Artery DiseasePercutaneous Coronary InterventionAcute Coronary Syndromes

Study of the Long-term Effects of P2Y12 Inhibitor Monotherapy and Coagulation Markers After Percutaneous Coronary Angioplasty.

RECRUITING
NCT07582835Phase PHASE3Cardiocentro TicinoStarted 2026-04-20
ClopidogrelTicagrelorPrasugrel
HIV Infections

A Clinical Trial to Evaluate the Safety and Immunogenicity of BG505 MD39.3, BG505 MD39.3 gp151, and BG505 MD39.3 gp151 CD4KO HIV Trimer mRNA Vaccines in Healthy, HIV-uninfected Adult Participants

ACTIVE NOT RECRUITING
NCT05217641Phase PHASE1National Institute of Allergy and Infectious Diseases (NIAID)Started 2022-02-11
BG505 MD39.3 mRNABG505 MD39.3 gp151 mRNABG505 MD39.3 gp151 CD4KO mRNA
Advanced Non-small Cell Lung Cancer, HER2 Mutation

A Study to Learn More About How Well Sevabertinib Works and How Safe it is Compared With Standard Treatment, in Participants Who Have Advanced Non-small Cell Lung Cancer (NSCLC) With Mutations of the Human Epidermal Growth Factor Receptor 2 (HER2)

RECRUITING
NCT06452277Phase PHASE3BayerStarted 2024-08-28
SevabertinibPembrolizumabCisplatin
Non-Small Cell Lung Cancer

A Study of Osimertinib With or Without Chemotherapy as 1st Line Treatment in Patients With Mutated Epidermal Growth Factor Receptor Non-Small Cell Lung Cancer (FLAURA2)

ACTIVE NOT RECRUITING
NCT04035486Phase PHASE3AstraZenecaStarted 2019-07-02
OsimertinibPemetrexed/CarboplatinPemetrexed/Cisplatin
Non Small Cell Lung Cancer (NSCLC)Colorectal Cancer (CRC)

Liquid BiOpsy, Lung and Colon Cancer in AndaluciA

ENROLLING BY INVITATION
NCT06997458Fundación Pública Andaluza para la Investigación Biomédica Andalucía OrientalStarted 2024-08-01
xF Tempus assay
Melanoma

A Clinical Trial of Three Study Medicines (Encorafenib, Binimetinib, and Pembrolizumab) in Patients With Advanced or Metastatic Melanoma

ACTIVE NOT RECRUITING
NCT04657991Phase PHASE3PfizerStarted 2021-01-15
EncorafenibBinimetinibPembrolizumab
Cancer Childhood

Ultrafast Whole Genome Sequencing for Childhood Cancer

RECRUITING
NCT07201038University of CambridgeStarted 2022-10-19