sapropterin (kuvan) Report issue

Small molecule Orphan Drug FDA Approved FDA First in Class Priority Review FDA

Active Ingredient History

  • Now
Sapropterin dihydrochloride, the active pharmaceutical ingredient in Kuvan Tablets, is a synthetic preparation of the dihydrochloride salt of naturally occurring tetrahydrobiopterin (BH4). Kuvan is indicated to reduce blood phenylalanine (Phe) levels in patients with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive Phenylketonuria (PKU). Kuvan is to be used in conjunction with a Phe-restricted diet. Kuvan has received orphan drug designation from both the U.S. Food and Drug Administration (FDA) and the European Medicines Agency (EMEA). Kuvan is a synthetic form of BH4, the cofactor for the enzyme phenylalanine hydroxylase (PAH). PAH hydroxylates Phe through an oxidative reaction to form tyrosine. In patients with PKU, PAH activity is absent or deficient. Treatment with BH4 can activate residual PAH enzyme, improve the normal oxidative metabolism of Phe, and decrease Phe levels in some patients.   NCATS

More Chemistry
  • Mechanism of Action:
  • Multi-specific: Missing data
  • Black Box: No
  • Availability: Prescription Only
  • Delivery Methods: Oral
  • Pro Drug: No

Drug Pricing (per unit)

United States

$18.3378 - $187.3580


$21.3471 - $106.9692
More Pricing Detail

Note: This drug pricing data is preliminary, incomplete, and may contain errors.

1-butanone, 1-(2,4,5-trihydroxyphenyl) | 2,4,5-trihydroxybutyrophenone | 2',4',5'-trihydroxybutyrophenone | 2-amino-6-(1,2-dihydroxypropyl)-5,6,7,8-tetrahydoro-4(1h)-pteridinone | 5,6,7,8-erythro-tetrahydrobiopterin | 5,6,7,8-tetrahydrobiopterin | 5,6,7,8-tetrahydrobiopterin, (s-(r*,s*))-isomer | 5,6,7,8-tetrahydrodictyopterin | 5,6,7,8-tetrahydro-l-erythrobiopterin | (−)-(6r)-2-amino-6-((1r,2s)-1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4(3h)-pteridinone | 6r-5,6,7,8-tetrahydrobiopterin | 6r-bh4 | 6r-l-5,6,7,8-tetrahydrobiopterin | (6r)-l-erythro-5,6,7,8-tetrahydrobiopterin | 6r-l-erythro-5,6,7,8-tetrahydrobiopterin | (6r)-l-erythro-tetrahydrobiopterin | bph4 | daproterin | d-threo-tetrahydrobiopterin | fenoptin | kuvan | phenoptin | phenylalanine hydroxylase cofactor | ravpal | r-thbp | sapropterin | sapropterina | sapropterin dihydrochloride | sapropterin hcl | sapropterin hydrochloride | sapropterinum | sun-0588 (shiratori) | t-1401 (biomarin) | tetrahydro-6-biopterin | tetrahydrobiopterin | thbp | trihydroxybutyrophenone


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