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tyrosinemia

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By Targets:	Amino acid Transporter (1) Apoptosis (1) Aurora Kinase (1) Bcl-2 Family (1) Endogenous Metabolite (4) HPPD (3) Isotope-Labeled Compounds (4) PKA (1) Reference Standards (2) STK33 (1)
By Research Areas:	Cancer (1) Cardiovascular Disease (2) Metabolic Disease (9)

Inhibitors & Agonists

Natural
Products

Isotope-Labeled Compounds

Cat. No.	Product Name	Target	Research Areas	Chemical Structure

HY-B0607

Nitisinone 2 Publications Verification NTBC; Nitisone; SC0735	HPPD	Metabolic Disease
Nitisinone is an orally active, competitive and reversible 4-hydroxyphenylpyruvate dioxygenase (4-HPPD) inhibitor with an IC₅₀ of 173 nM. Nitisinone promotes tyrosine accumulation in a dose-dependent manner. nitisinone can be used in studies of hereditary tyrosinemia type 1 (HT-1) (a rare genetic disorder) and albinism .

HY-W010040

4-Hydroxyphenylpyruvic acid 1 Publications Verification	Endogenous Metabolite	Cardiovascular Disease Metabolic Disease
4-Hydroxyphenylpyruvic acid is an intermediate in the metabolism of the amino acid phenylalanine. 4-Hydroxyphenylpyruvic acid is a substrate of 4-hydroxyphenylpyruvic acid dioxygenase (HPPA). 4-Hydroxyphenylpyruvic acid can be used in the study of HPD-related diseases, such as type III tyrosinemia and hawkinsinuria .

HY-13495

ML281 2 Publications Verification	STK33 PKA Aurora Kinase Bcl-2 Family Apoptosis	Metabolic Disease Cancer
ML281 is a highly selective inhibitor of serine/threonine kinase 33 (STK33) with an IC₅₀ value of 14 nM. ML281 shows 700-fold selectivity over PKA and 550-fold over AurB. ML281 exerts core mechanism by inhibiting STK33: in small cell lung cancer, ML281 downregulates RPS6/BAD signaling phosphorylation, induces apoptosis, and suppresses proliferation, invasion . ML281 reduces STK33-mediated 4-hydroxyphenylpyruvate dioxygenase (HPD) phosphorylation in tyrosinemia . ML281 is suitable for research on STK33 function, KRAS mutation-related cancers (pancreatic cancer, colon cancer, lung adenocarcinoma, etc.), small cell lung cancer, and tyrosinemia-related damage

HY-W010040R

4-Hydroxyphenylpyruvic acid (Standard)	Reference Standards Endogenous Metabolite	Cardiovascular Disease Metabolic Disease
4-Hydroxyphenylpyruvic acid (Standard) is the analytical standard of 4-Hydroxyphenylpyruvic acid (HY-W010040). This product is intended for research and analytical applications. 4-Hydroxyphenylpyruvic acid is an intermediate in the metabolism of the amino acid phenylalanine. 4-Hydroxyphenylpyruvic acid is a substrate of 4-hydroxyphenylpyruvic acid dioxygenase (HPPA). 4-Hydroxyphenylpyruvic acid can be used in the study of HPD-related diseases, such as type III tyrosinemia and hawkinsinuria .

HY-158161

SLC6A19-IN-1	Amino acid Transporter	Metabolic Disease
SLC6A19-IN-1 is a potent and highly selective inhibitor and transport corrector of human SLC6A19 (IC₅₀=47 nM). Furthermore, at a concentration of 35 μM, SLC6A19-IN-1 exhibits no activity against SLC1A5, SLC7A5, or SLC6A8. SLC6A19-IN-1 is applicable to research on phenylketonuria (PKU) and hyperphenylalaninemia. SLC6A19-IN-1 is also suitable for studies related to various metabolic disorders, including tyrosinemia, maple syrup urine disease, urea cycle disorders, and hyperammonemia .

HY-B0607R

Nitisinone (Standard) 2 Publications Verification NTBC (Standard); Nitisone (Standard); SC0735 (Standard)	HPPD Reference Standards	Metabolic Disease
Nitisinone (Standard) is the analytical standard of Nitisinone. This product is intended for research and analytical applications. Nitisinone is an orally active, competitive and reversible 4-hydroxyphenylpyruvate dioxygenase (4-HPPD) inhibitor with an IC₅₀ of 173 nM. Nitisinone promotes tyrosine accumulation in a dose-dependent manner. nitisinone can be used in studies of hereditary tyrosinemia type 1 (HT-1) (a rare genetic disorder) and albinism .

HY-W010589S1

H-Abu-OH-d2	Isotope-Labeled Compounds Endogenous Metabolite	Metabolic Disease
H-Abu-OH-d₂ is the deuterium labeled H-Abu-OH. H-Abu-OH, one of the three isomers of aminobutyric acid, is elevated in the plasma of children with with Reye's syndrome, tyrosinemia, homocystinuria, nonketotic hyperglycinemia, and ornithine transcarbamylase deficiency.

HY-W710927

L-2-Aminobutyric acid-d5	Isotope-Labeled Compounds Endogenous Metabolite	Metabolic Disease
L-2-Aminobutyric acid-d₅ is the deuterium labeled H-Abu-OH (HY-W010589). H-Abu-OH, one of the three isomers of aminobutyric acid, is elevated in the plasma of children with with Reye's syndrome, tyrosinemia, homocystinuria, nonketotic hyperglycinemia, and ornithine transcarbamylase deficiency.

HY-B0607S

Nitisinone-d3 NTBC-d₃; Nitisone-d₃; SC0735-d₃	Isotope-Labeled Compounds HPPD	Metabolic Disease
Nitisinone-d₃ (NTBC-d₃) is deuterium labeled Nitisinone. Nitisinone is an orally active, competitive and reversible 4-hydroxyphenylpyruvate dioxygenase (4-HPPD) inhibitor with an IC₅₀ of 173 nM. Nitisinone promotes tyrosine accumulation in a dose-dependent manner. nitisinone can be used in studies of hereditary tyrosinemia type 1 (HT-1) (a rare genetic disorder) and albinism .

HY-W777526

Nitisinone-13C6	Isotope-Labeled Compounds	Others
Nitisinone- ¹³C₆ is a ¹³C-labeled Nitisinone (HY-B0607). Nitisinone is an orally active, competitive and reversible 4-hydroxyphenylpyruvate dioxygenase (4-HPPD) inhibitor with an IC₅₀ of 173 nM. Nitisinone promotes tyrosine accumulation in a dose-dependent manner. nitisinone can be used in studies of hereditary tyrosinemia type 1 (HT-1) (a rare genetic disorder) and albinism .

Cat. No.	Product Name	Category	Target	Chemical Structure

HY-W010040

4-Hydroxyphenylpyruvic acid 1 Publications Verification	Structural Classification Monophenols Classification of Application Fields Ketones, Aldehydes, Acids Disease markers Phenols Endocrine diseases Metabolic Disease Nervous System Disorder Endogenous metabolite Disease Research Fields Source Classification	Endogenous Metabolite
4-Hydroxyphenylpyruvic acid is an intermediate in the metabolism of the amino acid phenylalanine. 4-Hydroxyphenylpyruvic acid is a substrate of 4-hydroxyphenylpyruvic acid dioxygenase (HPPA). 4-Hydroxyphenylpyruvic acid can be used in the study of HPD-related diseases, such as type III tyrosinemia and hawkinsinuria .

HY-W010040R

4-Hydroxyphenylpyruvic acid (Standard)	Structural Classification Monophenols Ketones, Aldehydes, Acids Disease markers Phenols Endocrine diseases Nervous System Disorder Endogenous metabolite Source Classification	Reference Standards Endogenous Metabolite
4-Hydroxyphenylpyruvic acid (Standard) is the analytical standard of 4-Hydroxyphenylpyruvic acid (HY-W010040). This product is intended for research and analytical applications. 4-Hydroxyphenylpyruvic acid is an intermediate in the metabolism of the amino acid phenylalanine. 4-Hydroxyphenylpyruvic acid is a substrate of 4-hydroxyphenylpyruvic acid dioxygenase (HPPA). 4-Hydroxyphenylpyruvic acid can be used in the study of HPD-related diseases, such as type III tyrosinemia and hawkinsinuria .

Cat. No.	Product Name	Chemical Structure

HY-W010589S1

H-Abu-OH-d2
H-Abu-OH-d₂ is the deuterium labeled H-Abu-OH. H-Abu-OH, one of the three isomers of aminobutyric acid, is elevated in the plasma of children with with Reye's syndrome, tyrosinemia, homocystinuria, nonketotic hyperglycinemia, and ornithine transcarbamylase deficiency.

HY-W710927

L-2-Aminobutyric acid-d5
L-2-Aminobutyric acid-d₅ is the deuterium labeled H-Abu-OH (HY-W010589). H-Abu-OH, one of the three isomers of aminobutyric acid, is elevated in the plasma of children with with Reye's syndrome, tyrosinemia, homocystinuria, nonketotic hyperglycinemia, and ornithine transcarbamylase deficiency.

HY-B0607S

Nitisinone-d3
Nitisinone-d₃ (NTBC-d₃) is deuterium labeled Nitisinone. Nitisinone is an orally active, competitive and reversible 4-hydroxyphenylpyruvate dioxygenase (4-HPPD) inhibitor with an IC₅₀ of 173 nM. Nitisinone promotes tyrosine accumulation in a dose-dependent manner. nitisinone can be used in studies of hereditary tyrosinemia type 1 (HT-1) (a rare genetic disorder) and albinism .

Nitisinone-d3

Nitisinone-d₃ (NTBC-d₃) is deuterium labeled Nitisinone. Nitisinone is an orally active, competitive and reversible 4-hydroxyphenylpyruvate dioxygenase (4-HPPD) inhibitor with an IC₅₀ of 173 nM. Nitisinone promotes tyrosine accumulation in a dose-dependent manner. nitisinone can be used in studies of hereditary tyrosinemia type 1 (HT-1) (a rare genetic disorder) and albinism .

HY-W777526

Nitisinone-13C6
Nitisinone- ¹³C₆ is a ¹³C-labeled Nitisinone (HY-B0607). Nitisinone is an orally active, competitive and reversible 4-hydroxyphenylpyruvate dioxygenase (4-HPPD) inhibitor with an IC₅₀ of 173 nM. Nitisinone promotes tyrosine accumulation in a dose-dependent manner. nitisinone can be used in studies of hereditary tyrosinemia type 1 (HT-1) (a rare genetic disorder) and albinism .

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BODIPY 493/503 purchased from MedChemExpress. Usage Cited in: Nat Commun. 2025 Feb 1;16(1):1241. [Abstract]

MedchemExpress Validation 01

Western blot analysis of extracts from THP-1(lane 2(20μg), Jurkat (lane 3(20μg) and NIH3T3(lane 4(20μg) using FOXO1A (HY-P80132) Rabbit mAb. Proteins were transferred to a PVDF membrane and blocked with 5% non-fat milk in TBST for 2 hour at room temperature. The primary antibody (1/1000) and Loading control antibody (Beta Actin, HY-P80438, 1/10000) was used in 5% non-fat milk in TBST at 4°C overnight. Goat Anti-Mouse/Rabbit IgG-HRP Secondary Antibody (1/10000) was used for 1 hour at room temperature. Western blot analysis of extracts from THP-1(lane 2(20μg), Jurkat (lane 3(20μg) and NIH3T3(lane 4(20μg) using FOXO1A (HY-P80132) Rabbit mAb. Proteins were transferred to a PVDF membrane and blocked with 5% non-fat milk in TBST for 2 hour at room temperature. The primary antibody (1/1000) and Loading control antibody (Beta Actin, HY-P80438, 1/10000) was used in 5% non-fat milk in TBST at 4°C overnight. Goat Anti-Mouse/Rabbit IgG-HRP Secondary Antibody (1/10000) was used for 1 hour at room temperature. Western blot analysis of extracts from THP-1(lane 2(20μg), Jurkat (lane 3(20μg) and NIH3T3(lane 4(20μg) using FOXO1A (HY-P80132) Rabbit mAb. Proteins were transferred to a PVDF membrane and blocked with 5% non-fat milk in TBST for 2 hour at room temperature. The primary antibody (1/1000) and Loading control antibody (Beta Actin, HY-P80438, 1/10000) was used in 5% non-fat milk in TBST at 4°C overnight. Goat Anti-Mouse/Rabbit IgG-HRP Secondary Antibody (1/10000) was used for 1 hour at room temperature.

BODIPY 493/503 purchased from MedChemExpress. Usage Cited in: Nat Commun. 2025 Feb 1;16(1):1241. [Abstract]

MedchemExpress Validation 01

MedchemExpress Validation 03

Western blot analysis of extracts from THP-1(lane 2(20μg), Jurkat (lane 3(20μg) and NIH3T3(lane 4(20μg) using FOXO1A (HY-P80132) Rabbit mAb. Proteins were transferred

MedchemExpress Validation 04

MedchemExpress Validation

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tyrosinemia

Inhibitors & Agonists

NaturalProducts

Isotope-Labeled Compounds

Natural
Products