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hyperammonemia

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By Targets:	Amino acid Transporter (1) Bacterial (1) Calcium Channel (1) Carbamoyl Phosphate Synthetase (CPS) (1) DNA/RNA Synthesis (1) Drug Intermediate (1) Drug Metabolite (1) Endogenous Metabolite (2) iGluR (1) Interleukin Related (1) NO Synthase (1) Ornithine decarboxylase (ODC) (1) Reference Standards (2)
By Research Areas:	Cancer (4) Endocrinology (1) Infection (1) Inflammation/Immunology (1) Metabolic Disease (3) Neurological Disease (1)

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Cat. No.	Product Name	Target	Research Areas	Chemical Structure

HY-B0711

Carglumic Acid 2 Publications Verification N-Carbamyl-L-glutamic acid	Carbamoyl Phosphate Synthetase (CPS)	Cancer
Carglumic acid (N-Carbamyl-L-glutamic acid), a functional analogue of N-acetylglutamate (NAG) and a carbamoyl phosphate synthetase 1 (CPS1) activator, is used to treat acute and chronic hyperammonemia associated with NAG synthase (NAGS) deficiency.

HY-B1172

Lactulose 1 Publications Verification 4-O-β-D-Galactopyranosyl-D-fructose	Bacterial Interleukin Related DNA/RNA Synthesis	Infection Inflammation/Immunology Endocrinology Cancer
Lactulose is an orally active galactose-fructose disaccharide. Lactulose suppresses upregulation of TNF-α and IL-6. Lactulose decreases the degree of DNA damage. Lactulose exhibits many of the properties of other oligosaccharides, including increasing the numbers of Bifidobacteria in feces. Lactulose restores the structure and composition of the intestinal microbiota, mitigates inflammation, and suppresses inflammatory tumorigenesis in mice with colitis-associated cancer. Lactulose can be used in the research of constipation .

HY-N0455B

L-Arginine (L-glutamate) 20+ Cited Publications (S)-(+)-Arginine (L-glutamate)	Endogenous Metabolite NO Synthase	Metabolic Disease
L-Arginine L-glutamate ((S)-(+)-Arginine L-glutamate) is the nitrogen donor for synthesis of nitric oxide. L-Arginine L-glutamate can be used for upper gastrointestinal hypofunction or dysfunction like functional dyspepsia research .

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-B0711R

Carglumic Acid (Standard) N-Carbamyl-L-glutamic acid (Standard)	Reference Standards Others	Cancer
Carglumic Acid (Standard) is the analytical standard of Carglumic Acid. This product is intended for research and analytical applications. Carglumic acid (N-Carbamyl-L-glutamic acid), a functional analogue of N-acetylglutamate (NAG) and a carbamoyl phosphate synthetase 1 (CPS1) activator, is used to treat acute and chronic hyperammonemia associated with NAG synthase (NAGS) deficiency.

HY-131275R

Imatinib Impurity E (Standard)	Drug Metabolite Reference Standards	Others
Carglumic Acid (Standard) is the analytical standard of Carglumic Acid. This product is intended for research and analytical applications. Carglumic acid (N-Carbamyl-L-glutamic acid), a functional analogue of N-acetylglutamate (NAG) and a carbamoyl phosphate synthetase 1 (CPS1) activator, is used to treat acute and chronic hyperammonemia associated with NAG synthase (NAGS) deficiency.

HY-B1713

Ornithine (±)-Ornithine	Endogenous Metabolite Ornithine decarboxylase (ODC) Drug Intermediate	Metabolic Disease Cancer
Ornithine ((±)-Ornithine) is a non-essential amino acid. Ornithine is an intermediate in urea cycle and acts as a substrate for these rate limiting enzymes ornithine transcarbomylase, ornithine aminotransferase and ornithine decarboxylase producing citrulline, proline and polyamines, respectively. Ornithine can be used in the research of hyperornithinemia, hyperammonemia, and cancer .

HY-131691

NMDAR blocker 1	iGluR Calcium Channel	Neurological Disease
NMDAR blocker 1 is an NMDA receptor channel blocker with an IC₅₀ of 5.0 μM. NMDAR blocker 1 exhibits fast on-off blockade kinetics and strong voltage dependence, and does not compete with glutamate or glycine. NMDAR blocker 1 prevents glutamate/NMDA-induced intracellular Ca ²⁺ overload, modulates the glutamate-nitric oxide-cGMP pathway. NMDAR blocker 1 prevents in vitro excitotoxic neurodegeneration of cultured cerebellar and hippocampal neurons. NMDAR blocker 1 attenuates excitotoxic insult in an mouse model of hyperammonemia-induced excitotoxicity. NMDAR blocker 1 can be used for the research of neurodegenerative diseases .

Cat. No.	Product Name

HY-L189

Amino Acid Metabolism Compound Library	335 compounds
Amino acids, as one of the most fundamental organic compounds in living organisms, serve not only as the basic building blocks of proteins but also but also undertake the functions of energy supply, neurotransmitter synthesis, and maintenance of internal environment stability.Amino acid metabolic enzymes are a class of enzymes involved in the metabolic processes of amino acids, catalyzing their synthesis, breakdown, transformation, and interactions with other metabolic pathways. Abnormalities in amino acid metabolic enzymes can lead to various metabolic diseases, such as phenylketonuria and hyperammonemia, etc. Therefore, actively exploring and regulating the processes of amino acid metabolism is crucial for the development of drugs related to these diseases. MCE designs a unique collection of 335 small molecules target amino acid metabolizing enzymes, which is an important tool for studying studying amino acid metabolism processes or metabolism-related drug development.

Amino Acid Metabolism Compound Library

335 compounds

Amino acids, as one of the most fundamental organic compounds in living organisms, serve not only as the basic building blocks of proteins but also but also undertake the functions of energy supply, neurotransmitter synthesis, and maintenance of internal environment stability.Amino acid metabolic enzymes are a class of enzymes involved in the metabolic processes of amino acids, catalyzing their synthesis, breakdown, transformation, and interactions with other metabolic pathways. Abnormalities in amino acid metabolic enzymes can lead to various metabolic diseases, such as phenylketonuria and hyperammonemia, etc. Therefore, actively exploring and regulating the processes of amino acid metabolism is crucial for the development of drugs related to these diseases.

MCE designs a unique collection of 335 small molecules target amino acid metabolizing enzymes, which is an important tool for studying studying amino acid metabolism processes or metabolism-related drug development.

Lactulose 1 Publications Verification 4-O-β-D-Galactopyranosyl-D-fructose	Structural Classification Polysaccharides Classification of Application Fields Other Diseases Endogenous metabolite Disease Research Fields Saccharides Source Classification	Bacterial Interleukin Related DNA/RNA Synthesis
Lactulose is an orally active galactose-fructose disaccharide. Lactulose suppresses upregulation of TNF-α and IL-6. Lactulose decreases the degree of DNA damage. Lactulose exhibits many of the properties of other oligosaccharides, including increasing the numbers of Bifidobacteria in feces. Lactulose restores the structure and composition of the intestinal microbiota, mitigates inflammation, and suppresses inflammatory tumorigenesis in mice with colitis-associated cancer. Lactulose can be used in the research of constipation .

L-Arginine (L-glutamate) 20+ Cited Publications (S)-(+)-Arginine (L-glutamate)	Ketones, Aldehydes, Acids Endogenous metabolite Source Classification	Endogenous Metabolite NO Synthase
L-Arginine L-glutamate ((S)-(+)-Arginine L-glutamate) is the nitrogen donor for synthesis of nitric oxide. L-Arginine L-glutamate can be used for upper gastrointestinal hypofunction or dysfunction like functional dyspepsia research .

Carglumic Acid (Standard) N-Carbamyl-L-glutamic acid (Standard)	Human Gut Microbiota Metabolites Microorganisms Endogenous metabolite Source Classification	Reference Standards Others
Carglumic Acid (Standard) is the analytical standard of Carglumic Acid. This product is intended for research and analytical applications. Carglumic acid (N-Carbamyl-L-glutamic acid), a functional analogue of N-acetylglutamate (NAG) and a carbamoyl phosphate synthetase 1 (CPS1) activator, is used to treat acute and chronic hyperammonemia associated with NAG synthase (NAGS) deficiency.

Ornithine (±)-Ornithine	Structural Classification Human Gut Microbiota Metabolites Ketones, Aldehydes, Acids Endogenous metabolite Source Classification	Endogenous Metabolite Ornithine decarboxylase (ODC) Drug Intermediate
Ornithine ((±)-Ornithine) is a non-essential amino acid. Ornithine is an intermediate in urea cycle and acts as a substrate for these rate limiting enzymes ornithine transcarbomylase, ornithine aminotransferase and ornithine decarboxylase producing citrulline, proline and polyamines, respectively. Ornithine can be used in the research of hyperornithinemia, hyperammonemia, and cancer .

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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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hyperammonemia

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