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phenylketonuria

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製品番号 製品名 Target 研究分野 構造式
  • 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

    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-30220

    L-(-)-3-Phenyllactic Acid

    Endogenous Metabolite Fungal Infection Metabolic Disease
    (S)-2-Hydroxy-3-phenylpropanoic acid is the L-configuration of 2-Hydroxy-3-phenylpropanoic acid, and its level is closely related to some diseases, such as phenylketonuria .
    (S)-2-Hydroxy-3-phenylpropanoic acid
  • HY-W015590

    Drug Metabolite Fungal Infection Metabolic Disease
    2-Hydroxyphenylacetic acid is a metabolite of Phenylalanine and Tyrosine, as well as a product of the biotransformation of Phenylacetonitrile by marine fungi. 2-Hydroxyphenylacetic acid acts as a potential biomarker for food. 2-Hydroxyphenylacetic acid slightly induces the expression of green fluorescent protein. 2-Hydroxyphenylacetic acid is used in the research of phenylketonuria and related hyperphenylalaninemia .
    2-Hydroxyphenylacetic acid
  • HY-A0124

    (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-A0124AR

    (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-162864

    Amino acid Transporter Metabolic Disease
    JX237 is an inhibitor of the epithelial neutral amino acid transporter B 0AT1 (SLC6A19) with an IC50 value of 31 nM. SLC6A19 is the main transporter for the absorption of neutral amino acids in the intestines and their reabsorption in the kidneys. By inhibiting B 0AT1, JX237 can be used for the study of disorders of amino acid metabolism .
    JX237
  • HY-158161

    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-P2964

    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-P10563

    BHV-1100

    CD38 Cancer
    Noraramtide (BHV-1100) is an antibody recruitment molecule. Noraramtide can specifically bind to CD38 molecules to recruit natural killer (NK) cells. Noraramtide enhances the ability of NK cells to kill tumor cells through antibody-dependent cellular cytotoxicity (ADCC). This mechanism allows NK cells to more effectively recognize and eliminate tumor cells while avoiding mutual killing between NK cells. Noraramtide can be used for the study of autologous cancer immunity .
    Noraramtide
  • HY-144856S1

    (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-P3350

    Bacterial Infection
    LS-BF1 is a stable and low toxic cationic antimicrobial peptide. LS-BF1 displays broad spectrum of antibacterial activity, including the challenging ESKAPE pathogens, by cell membrane disruptive mechanism. LS-BF1 shows good in vivo efficacy for elimination of bacteria in a mouse infection model[1].
    LS-BF1
  • HY-179107

    Amino acid Transporter Metabolic Disease
    SLC6A19-IN-3 (Compound 83-P1-P2) is a potent, selective and orally active SLC6A19 inhibitor with an IC50 of 28 nM. SLC6A19-IN-3 can block SLC6A19-mediated transmembrane transport of phenylalanine, reducing intestinal absorption of phenylalanine from food and renal tubular reabsorption of phenylalanine. SLC6A19-IN-3 can be used for the research of metabolic disease, such as phenylketonuria .
    SLC6A19-IN-3
  • HY-P2951

    Biochemical Assay Reagents Metabolic Disease
    Phenylalanine ammonia-lyase is a non-mammalian enzyme converting phenylalanine into trans-cinnamic acid and ammonia. Phenylalanine ammonia-lyase can be used in the research of phenylketonuria .
    Phenylalanine ammonia-lyase
  • HY-176873

    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-105168

    Endothelin Receptor Cardiovascular Disease
    TAK 044 is an antagonist of Endothelin Receptor. TAK 044 strongly inhibits ET-induced deterioration in various animal models. TAK 044 can be used in study ET-related diseases such as acute myocardial infarction,acute renal failure, acute hepatic malfunction, and subarachnoid hemorrhage .
    TAK 044
  • HY-W015590R

    Reference Standards Drug Metabolite Fungal Infection Metabolic Disease
    2-Hydroxyphenylacetic acid (Standard) is an analytical standard for 2-Hydroxyphenylacetic acid (HY-W015590). This product is intended for research and analytical applications. 2-Hydroxyphenylacetic acid is a metabolite of Phenylalanine and Tyrosine, and is also a product of the biotransformation of Phenylacetonitrile by marine fungi. 2-Hydroxyphenylacetic acid can be used as a potential biomarker for food. 2-Hydroxyphenylacetic acid can be used in the study of phenylketonuria and related hyperphenylalaninemias .
    2-Hydroxyphenylacetic acid (Standard)
  • HY-30220R

    L-(-)-3-Phenyllactic Acid (Standard)

    Endogenous Metabolite Fungal Reference Standards Infection Metabolic Disease
    (S)-2-Hydroxy-3-phenylpropanoic acid (Standard) is the analytical standard of (S)-2-Hydroxy-3-phenylpropanoic acid. This product is intended for research and analytical applications. (S)-2-Hydroxy-3-phenylpropanoic acid is the L-configuration of 2-Hydroxy-3-phenylpropanoic acid, and its level is closely related to some diseases, such as phenylketonuria .
    (S)-2-Hydroxy-3-phenylpropanoic acid (Standard)
  • HY-W753181

    L-(-)-3-Phenyllactic Acid-13C9

    Isotope-Labeled Compounds Fungal Endogenous Metabolite Metabolic Disease
    (S)-2-Hydroxy-3-phenylpropanoic acid- 13C9 (L-(-)-3-Phenyllactic Acid- 13C9) is the 13C-labeled (S)-2-Hydroxy-3-phenylpropanoic acid (HY-30220). (S)-2-Hydroxy-3-phenylpropanoic acid is the L-configuration of 2-Hydroxy-3-phenylpropanoic acid, and its level is closely related to some diseases, such as phenylketonuria .
    (S)-2-Hydroxy-3-phenylpropanoic acid-13C9
  • HY-179581

    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
  • HY-P4948

    Biochemical Assay Reagents Others
    Coumarin-phalloidin is a kind of phalloidin labeled with Coumarin (HY-N0709). Coumarin-phalloidin is a new type of actin probe that can be used for triple immunofluorescence microscopic observation of the cell skeleton .
    Coumarin-Phalloidin

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