8 Results for "

maple syrup urine disease

" in MedChemExpress (MCE) Product Catalog:
Products (8)

8 Results for "maple syrup urine disease" in MCE Product Catalog:

1
1 Cited Publications
Cat. No.: HY-W012722
CAS No.: 816-66-0
Synonyms: α-Ketoisocaproic acid
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 .
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Cat. No.: HY-W015874
CAS No.: 3739-30-8
2-Hydroxy-2-methylbutanoic acid is a branched-chain fatty acid. 2-Hydroxy-2-methylbutanoic acid accumulates at high levels in the culture supernatants of colorectal cancer-associated bacteria. 2-Hydroxy-2-methylbutanoic acid is also present in the urine of patients with 2-hydroxyglutaric aciduria and maple syrup urine disease .
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Cat. No.: HY-164804
CAS No.: 70190-98-6
Target:  

Endogenous Metabolite

Research Areas:  

Metabolic Disease

N-Lactylvaline is an endogenous N-lactoyl-amino acid formed from L-lactic acid and L-valine. N-Lactylvaline has been detected in intermediate maple syrup urine disease .
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Cat. No.: HY-164805
CAS No.: 112757-17-2
Target:  

Endogenous Metabolite

Research Areas:  

Others Metabolic Disease

N-Lactylleucine is an endogenous N-lactoyl-amino acid formed from L-lactic acid and L-leucine. N-Lactylleucine has been detected in intermediate maple syrup urine disease .
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Cat. No.: HY-158161
CAS No.: 3032920-98-9
Research Areas:  

Metabolic Disease

JN-170 is a potent and highly selective inhibitor and transport corrector of human SLC6A19 (IC50=47 nM). Furthermore, at a concentration of 35 μM, JN-170 exhibits no activity against SLC1A5, SLC7A5, or SLC6A8. JN-170 is applicable to research on phenylketonuria (PKU) and hyperphenylalaninemia. JN-170 is also suitable for studies related to various metabolic disorders, including tyrosinemia, maple syrup urine disease, urea cycle disorders, and hyperammonemia .
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Cat. No.: HY-164805S
N-Lactylleucine- 13C6, 15N is the 15N- and 13C-labeled N-Lactylleucine (HY-164805). N-Lactylleucine is an endogenous N-lactoyl-amino acid formed from L-lactic acid and L-leucine. N-Lactylleucine has been detected in intermediate maple syrup urine disease .
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Cat. No.: HY-W015874R
CAS No.: 3739-30-8
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.
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Cat. No.: HY-P74207
Purity:  ≥ 95%, as determined by reducing SDS-PAGE.
Synonyms: DBT; Dihydrolipoamide Branched Chain Transacylase (E2 Component Of Branched Chain Keto Acid Dehydrogenase Complex; maple syrup urine disease); Dihydrolipoamide Branched Chain Transacylase E2; Lipoamide Acyltransferase Component Of Mitochondrial Branched-C
Species:  
Human
Source:  
Sf9 insect cells
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