Tetrahydrobiopterin
Brand names,
Tetrahydrobiopterin
Analogs
Tetrahydrobiopterin
Brand Names Mixture
Tetrahydrobiopterin
Chemical_Formula
C9H15N5O3
Tetrahydrobiopterin
RX_link
No information avaliable
Tetrahydrobiopterin
fda sheet
Tetrahydrobiopterin
msds (material safety sheet)
Tetrahydrobiopterin
Synthesis Reference
No information avaliable
Tetrahydrobiopterin
Molecular Weight
241.247 g/mol
Tetrahydrobiopterin
Melting Point
250-255 oC (hydrochloride salt)
Tetrahydrobiopterin
H2O Solubility
>20 mg/mL (dichloride salt)
Tetrahydrobiopterin
State
Solid
Tetrahydrobiopterin
LogP
-1.783
Tetrahydrobiopterin
Dosage Forms
No information avaliable
Tetrahydrobiopterin
Indication
For the treatment of tetrahydrobiopterin (BH4) deficiency.
Tetrahydrobiopterin
Pharmacology
Tetrahydrobiopterin (BH4) is used to convert several amino acids, including phenylalanine, to other essential molecules in the body including neurotransmitters. Tetrahydrobiopterin deficiency can be caused by mutations in GTP cyclohydrolase 1 (GCH1), 6-pyruvoyl-tetrahydropterin synthase/dimerization cofactor of hepatocyte nuclear factor 1 alpha (PCBD1), 6-pyruvoyltetrahydropterin synthase (PTS), and quinoid dihydropteridine reductase (QDPR) genes. These genes make the enzymes that are critical for producing and recycling tetrahydrobiopterin. If one of the enzymes fails to function correctly because of a gene mutation, little or no tetrahydrobiopterin is produced. As a result, phenylalanine from the diet builds up in the bloodstream and other tissues and can damage nerve cells in the brain. High levels of phenylalanine can result in signs and symptoms ranging from temporary low muscle tone to mental retardation, movement disorders, difficulty swallowing, seizures, behavioral problems, progressive problems with development, and an inability to control body temperature.
Tetrahydrobiopterin
Absorption
No information avaliable
Tetrahydrobiopterin
side effects and Toxicity
No information avaliable
Tetrahydrobiopterin
Patient Information
No information avaliable
Tetrahydrobiopterin
Organisms Affected
Humans and other mammals