14 Results for "

PKU

" in MedChemExpress (MCE) Product Catalog:
Products (14)

14 Results for "PKU" in MCE Product Catalog:

3
3 Cited Publications
Cat. No.: HY-A0124A
CAS No.: 69056-38-8
Synonyms: (6R)-BH4 dihydrochloride; (6R)-Tetrahydro-L-biopterin dihydrochloride
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) .
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Cat. No.: HY-156682
CAS No.: 2837993-05-0
Purity:  99.00%
Research Areas:  

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) .
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Cat. No.: HY-A0124AR
CAS No.: 69056-38-8
Synonyms: (6R)-BH4 dihydrochloride (Standard); (6R)-Tetrahydro-L-biopterin dihydrochloride (Standard)
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) .
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Cat. No.: HY-A0124
CAS No.: 62989-33-7
Synonyms: (6R)-BH4; (6R)-Tetrahydro-L-biopterin
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) .
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Cat. No.: HY-P2964
CAS No.: 69403-12-9
Research Areas:  

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) .
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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-144856S1
CAS No.: 2747956-15-4
Synonyms: (6R)-BH4-d3; (6R)-Tetrahydro-L-biopterin-d3
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) .
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Cat. No.: HY-176873
CAS No.: 3102931-05-2
Research Areas:  

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) .
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Cat. No.: HY-RS09979
Research Areas:  

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.

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Cat. No.: HY-P88444
Synonyms: HsHPK; PKU-ALPHA

Host:  

Mouse

Application:  

IHC-P, FC

Reactivity:  

Human, Mouse, Rat

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Cat. No.: HY-P88444A
Synonyms: HsHPK; PKU-ALPHA

Host:  

Mouse

Application:  

IHC-P, FC

Reactivity:  

Human, Mouse, Rat

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Cat. No.: HY-P702737
Purity:  ≥ 80%, as determined by reducing SDS-PAGE.
Synonyms: TLK2; tousled-like kinase 2; serine/threonine-protein kinase tousled-like 2; MGC44450; PKU ALPHA; serine/threonine kinase; PKU-ALPHA
Species:  
Source:  
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Cat. No.: HY-P76676
Purity:  ≥ 85%, as determined by reducing SDS-PAGE.
Synonyms: Serine/threonine-protein kinase tousled-like 1; PKU-beta; Tousled-like kinase 1; TLK1; KIAA0137
Species:  
Source:  
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Cat. No.: HY-179581
CAS No.: 2962069-39-0
Research Areas:  

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 .
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