Maple Syrup Urine Disease

By Targets:	Autophagy (1) Endogenous Metabolite (9) Isotope-Labeled Compounds (1) mTOR (1) Reference Standards (1) SOD (1)
By Research Areas:	Metabolic Disease (8) Neurological Disease (1)

Cat. No.	Product Name	Target	Research Areas	Chemical Structure

HY-164805

N-Lactylleucine	Endogenous Metabolite	Others
N-Lactylleucine is an endogenous metabolite that can be identified in patients with the intermediate type of maple syrup urine disease .

HY-164804

N-Lactylvaline	Endogenous Metabolite	Metabolic Disease
N-Lactylvaline is an endogenous metabolite that can be identified in patients with the intermediate type of maple syrup urine disease .

HY-W015466

Acetylvaline	Endogenous Metabolite	Metabolic Disease
Acetylvaline is an amino acid derivative belonging to the N-acetylated amino acid family. Acetylvaline can be detected in small quantities in the urine of healthy individuals but shows significantly abnormal excretion in the urine of patients with maple syrup urine disease (MSUD). Acetylvaline can be used to detect MSUD .

HY-W015874

2-Hydroxy-2-methylbutanoic acid	Endogenous Metabolite	Metabolic Disease
2-Hydroxy-2-methylbutanoic acid, an unusual metabolite, is associated with 2-hydroxyglutaric aciduria and maple syrup urine disease.

HY-W012722

4-Methyl-2-oxopentanoic acid 1 Publications Verification α-Ketoisocaproic acid	Endogenous Metabolite Autophagy mTOR SOD	Neurological Disease Metabolic Disease
4-Methyl-2-oxopentanoic acid (α-Ketoisocaproic acid) is a metabolite of L-leucine and is involved in energy metabolism. 4-Methyl-2-oxopentanoic acid increases endoplasmic reticulum stress, promotes lipid accumulation in preadipocytes and insulin resistance by impairing mTOR and autophagy signaling pathways. 4-Methyl-2-oxopentanoic acid also causes oxidative damage, leading to cognitive deficits, inhibits α-ketoglutarate dehydrogenase activity, acts as an oxidative phosphorylation uncoupler and metabolic inhibitor. 4-Methyl-2-oxopentanoic acid acts as a nutrient signal and stimulates skeletal muscle protein synthesis. 4-Methyl-2-oxopentanoic acid can be used in the study of maple syrup urine disease .

HY-W015466S

Acetylvaline-15N	Endogenous Metabolite	Metabolic Disease
Acetylvaline- ¹⁵N is the ¹⁵N-labeled Acetylvaline (HY-W015466). Acetylvaline is an amino acid derivative belonging to the N-acetylated amino acid family. Acetylvaline can be detected in small quantities in the urine of healthy individuals but shows significantly abnormal excretion in the urine of patients with maple syrup urine disease (MSUD). Acetylvaline can be used to detect MSUD.

HY-W015466R

Acetylvaline (Standard)	Endogenous Metabolite	Metabolic Disease
Acetylvaline (Standard) is an analytical standard for acetylvaline (HY-W015466). This product is intended for research and analytical applications. Acetylvaline is an amino acid derivative that belongs to the N-acetylated amino acid family. Acetylvaline is detected in low levels in the urine of healthy individuals, but significant abnormal excretion is observed in the urine of individuals with maple syrup urine disease (MSUD). Acetylvaline can be used to detect MSUD.

HY-W015466S1

Acetylvaline-13C2	Isotope-Labeled Compounds Endogenous Metabolite	Metabolic Disease
Acetylvaline-13C2 is the ¹³C-labeled Acetylvaline (HY-W015466). Acetylvaline is an amino acid derivative belonging to the N-acetylated amino acid family. Acetylvaline can be detected in small quantities in the urine of healthy individuals but shows significantly abnormal excretion in the urine of patients with maple syrup urine disease (MSUD). Acetylvaline can be used to detect MSUD.

HY-W015874R

2-Hydroxy-2-methylbutanoic acid (Standard)	Reference Standards Endogenous Metabolite	Metabolic Disease
2-Hydroxy-2-methylbutanoic acid (Standard) is the analytical standard of 2-Hydroxy-2-methylbutanoic acid. This product is intended for research and analytical applications. 2-Hydroxy-2-methylbutanoic acid, an unusual metabolite, is associated with 2-hydroxyglutaric aciduria and maple syrup urine disease.

Cat. No.	Product Name	Target	Research Area

HY-164805

N-Lactylleucine	Endogenous Metabolite	Others
N-Lactylleucine is an endogenous metabolite that can be identified in patients with the intermediate type of maple syrup urine disease .

HY-164804

N-Lactylvaline	Endogenous Metabolite	Metabolic Disease
N-Lactylvaline is an endogenous metabolite that can be identified in patients with the intermediate type of maple syrup urine disease .

Cat. No.	Product Name	Category	Target	Chemical Structure

HY-W015466

Acetylvaline	Structural Classification Classification of Application Fields Ketones, Aldehydes, Acids Source classification Metabolic Disease Endogenous metabolite Disease Research Fields	Endogenous Metabolite
Acetylvaline is an amino acid derivative belonging to the N-acetylated amino acid family. Acetylvaline can be detected in small quantities in the urine of healthy individuals but shows significantly abnormal excretion in the urine of patients with maple syrup urine disease (MSUD). Acetylvaline can be used to detect MSUD .

HY-W015874

2-Hydroxy-2-methylbutanoic acid	Structural Classification Other disease Classification of Application Fields Ketones, Aldehydes, Acids Source classification Disease markers Metabolic Disease Endogenous metabolite Disease Research Fields	Endogenous Metabolite
2-Hydroxy-2-methylbutanoic acid, an unusual metabolite, is associated with 2-hydroxyglutaric aciduria and maple syrup urine disease.

HY-W012722

4-Methyl-2-oxopentanoic acid 1 Publications Verification α-Ketoisocaproic acid	Structural Classification Human Gut Microbiota Metabolites Immune System Disorder Microorganisms Ketones, Aldehydes, Acids Source classification Disease markers Endogenous metabolite	Endogenous Metabolite Autophagy mTOR SOD
4-Methyl-2-oxopentanoic acid (α-Ketoisocaproic acid) is a metabolite of L-leucine and is involved in energy metabolism. 4-Methyl-2-oxopentanoic acid increases endoplasmic reticulum stress, promotes lipid accumulation in preadipocytes and insulin resistance by impairing mTOR and autophagy signaling pathways. 4-Methyl-2-oxopentanoic acid also causes oxidative damage, leading to cognitive deficits, inhibits α-ketoglutarate dehydrogenase activity, acts as an oxidative phosphorylation uncoupler and metabolic inhibitor. 4-Methyl-2-oxopentanoic acid acts as a nutrient signal and stimulates skeletal muscle protein synthesis. 4-Methyl-2-oxopentanoic acid can be used in the study of maple syrup urine disease .

HY-W015466R

Acetylvaline (Standard)	Structural Classification Ketones, Aldehydes, Acids Source classification Endogenous metabolite	Endogenous Metabolite
Acetylvaline (Standard) is an analytical standard for acetylvaline (HY-W015466). This product is intended for research and analytical applications. Acetylvaline is an amino acid derivative that belongs to the N-acetylated amino acid family. Acetylvaline is detected in low levels in the urine of healthy individuals, but significant abnormal excretion is observed in the urine of individuals with maple syrup urine disease (MSUD). Acetylvaline can be used to detect MSUD.

HY-W015874R

2-Hydroxy-2-methylbutanoic acid (Standard)	Structural Classification Other disease Ketones, Aldehydes, Acids Source classification Disease markers Endogenous metabolite	Reference Standards Endogenous Metabolite
2-Hydroxy-2-methylbutanoic acid (Standard) is the analytical standard of 2-Hydroxy-2-methylbutanoic acid. This product is intended for research and analytical applications. 2-Hydroxy-2-methylbutanoic acid, an unusual metabolite, is associated with 2-hydroxyglutaric aciduria and maple syrup urine disease.

Cat. No.	Product Name	Chemical Structure

HY-W015466S

Acetylvaline-15N
Acetylvaline- ¹⁵N is the ¹⁵N-labeled Acetylvaline (HY-W015466). Acetylvaline is an amino acid derivative belonging to the N-acetylated amino acid family. Acetylvaline can be detected in small quantities in the urine of healthy individuals but shows significantly abnormal excretion in the urine of patients with maple syrup urine disease (MSUD). Acetylvaline can be used to detect MSUD.

Acetylvaline-15N

Acetylvaline- ¹⁵N is the ¹⁵N-labeled Acetylvaline (HY-W015466). Acetylvaline is an amino acid derivative belonging to the N-acetylated amino acid family. Acetylvaline can be detected in small quantities in the urine of healthy individuals but shows significantly abnormal excretion in the urine of patients with maple syrup urine disease (MSUD). Acetylvaline can be used to detect MSUD.

HY-W015466S1

Acetylvaline-13C2
Acetylvaline-13C2 is the ¹³C-labeled Acetylvaline (HY-W015466). Acetylvaline is an amino acid derivative belonging to the N-acetylated amino acid family. Acetylvaline can be detected in small quantities in the urine of healthy individuals but shows significantly abnormal excretion in the urine of patients with maple syrup urine disease (MSUD). Acetylvaline can be used to detect MSUD.

Acetylvaline-13C2

Acetylvaline-13C2 is the ¹³C-labeled Acetylvaline (HY-W015466). Acetylvaline is an amino acid derivative belonging to the N-acetylated amino acid family. Acetylvaline can be detected in small quantities in the urine of healthy individuals but shows significantly abnormal excretion in the urine of patients with maple syrup urine disease (MSUD). Acetylvaline can be used to detect MSUD.

MCE Japan Authorized Agent ナミキ商事株式会社コスモ・バイオ株式会社重松貿易株式会社
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