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Results for "

PKU

" in MedChemExpress (MCE) Product Catalog:

10

Inhibitors & Agonists

1

Natural
Products

2

Recombinant Proteins

1

Isotope-Labeled Compounds

2

Antibodies

1

Oligonucleotides

Cat. No. Product Name Target Research Areas Chemical Structure
  • HY-A0124A
    Sapropterin dihydrochloride
    2 Publications Verification

    (6R)-BH4 dihydrochloride; (6R)-Tetrahydro-L-biopterin dihydrochloride

    		 Endogenous Metabolite Infection Metabolic Disease Inflammation/Immunology
    Sapropterin ((6R)-BH4; (6R)-Tetrahydro-L-biopterin) dihydrochloride is an orally active phenylalanine hydroxylase (PAH) cofactor, which is effective in reducing blood phenylalanine concentrations. Sapropterin dihydrochloride can be used in study of phenylketonuria (PKU) phenylketonuria. Sapropterin dihydrochloride can aggravate experimental autoimmune encephalomyelitis (EAE) .
    Sapropterin dihydrochloride
  • HY-156682
    JNT-517

    		Amino acid Transporter Metabolic Disease
    JNT-517 is an orally active, selective SLC6A19 allosteric inhibitor with an IC50 of 47 nM for human SLC6A19. JNT-517 can be used for the study of phenylketonuria (PKU) .
    JNT-517
  • HY-A0124AR
    Sapropterin dihydrochloride (Standard)

    (6R)-BH4 dihydrochloride (Standard); (6R)-Tetrahydro-L-biopterin dihydrochloride (Standard)

    		 Endogenous Metabolite Reference Standards Infection Metabolic Disease Inflammation/Immunology
    Sapropterin ((6R)-BH4; (6R)-Tetrahydro-L-biopterin) dihydrochloride (Standard) is the analytical standard of Sapropterin dihydrochloride (HY-A0124A) . This product is intended for research and analytical applications. Sapropterin dihydrochloride is an orally active phenylalanine hydroxylase (PAH) cofactor, which is effective in reducing blood phenylalanine concentrations. Sapropterin dihydrochloride can be used in study of phenylketonuria (PKU) phenylketonuria. Sapropterin dihydrochloride can aggravate experimental autoimmune encephalomyelitis (EAE) .
    Sapropterin dihydrochloride (Standard)
  • HY-A0124
    Sapropterin

    (6R)-BH4; (6R)-Tetrahydro-L-biopterin

    		 Endogenous Metabolite Infection Metabolic Disease Inflammation/Immunology
    Sapropterin ((6R)-BH4; (6R)-Tetrahydro-L-biopterin) is an orally active phenylalanine hydroxylase (PAH) cofactor, which is effective in reducing blood phenylalanine concentrations. Sapropterin can be used in study of phenylketonuria (PKU) phenylketonuria. Sapropterin can aggravate experimental autoimmune encephalomyelitis (EAE) .
    Sapropterin
  • HY-P2964
    Phenylalanine dehydrogenase

    		Endogenous Metabolite Metabolic Disease
    Phenylalanine dehydrogenase is an NAD +-dependent oxidoreductase targeting L-phenylalanine. Phenylalanine dehydrogenase catalyzes deamination to phenylpyruvate and NADH as part of amino acid metabolism regulation. Phenylalanine dehydrogenase is promising for research of phenylketonuria (PKU) .
    Phenylalanine dehydrogenase
  • 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 .
    SLC6A19-IN-1
  • HY-144856S1
    Sapropterin-d3

    (6R)-BH4-d3; (6R)-Tetrahydro-L-biopterin-d3

    		 Isotope-Labeled Compounds Endogenous Metabolite Metabolic Disease
    Sapropterin-d3 ((6R)-BH4-d3) is deuterium labeled Sapropterin. Sapropterin ((6R)-BH4) is an orally active phenylalanine hydroxylase (PAH) cofactor, which is effective in reducing blood phenylalanine concentrations. Sapropterin can be used in study of phenylketonuria (PKU) .
    Sapropterin-d3
  • HY-176873
    SLC6A19-IN-2

    		Amino acid Transporter Metabolic Disease
    SLC6A19-IN-2 (Example 4) is a potent SLC6A19 inhibitor with an IC50 of 14 nM. SLC6A19-IN-2 can be used for the study of metabolic diseases such as nonalcoholic steatohepatitis (NASH), nonalcoholic fatty liver disease (NAFLD) and phenylketonuria (PKU) .
    SLC6A19-IN-2
  • HY-RS09979
    PAH Human Pre-designed siRNA Set A

    		Small Interfering RNA (siRNA) Others

    PAH Human Pre-designed siRNA Set A contains three designed siRNAs for PAH gene (Human), as well as a negative control, a positive control, and a FAM-labeled negative control.

    PAH Human Pre-designed siRNA Set A
    PAH Human Pre-designed siRNA Set A
  • HY-179581
    SLC6A19-IN-4

    		Amino acid Transporter Metabolic Disease
    SLC6A19-IN-4 is an allosteric-competitive and orally active B 0AT1 inhibitor. SLC6A19-IN-4 inhibits both human and mouse B 0AT1 with IC50 values of 513 nM and 295 nM, respectively. SLC6A19-IN-4 exhibits excellent metabolic stability. SLC6A19-IN-4 significantly increases urinary phenylalanine (Phe) excretion and reduces plasma Phe levels through dual inhibition of B 0AT1 in both the intestine (reducing absorption) and kidney (promoting excretion) in vivo. SLC6A19-IN-4 can be used for phenylketonuria (PKU) and other disorders involving SLC6-family transporters research .
    SLC6A19-IN-4

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