-
Tetrahydrobiopterin (BH4, THB), also
known as
sapropterin (INN), is a
cofactor of the
three aromatic amino acid
hydroxylase enzymes, used in the degradation...
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Tetrahydrobiopterin deficiency (THBD, BH4D) is a rare
metabolic disorder that
increases the
blood levels of phenylalanine.
Phenylalanine is an
amino acid...
- ****emblies of phenylalanine. A
rarer form of
hyperphenylalaninemia is
tetrahydrobiopterin deficiency,
which occurs when the PAH
enzyme is normal, and a defect...
- does so
using molecular oxygen (O2), as well as iron (Fe2+) and
tetrahydrobiopterin as cofactors. L-DOPA is a
precursor for dopamine, which, in turn...
-
insertion of
molybdate into MPT to form Moco (molybdenum cofactor).
Tetrahydrobiopterin, the
major unconjugated pterin in vertebrates, is
involved in three...
-
aromatic amino acid hydroxylases, a
class of
monooxygenase that uses
tetrahydrobiopterin (BH4, a
pteridine cofactor) and a non-heme iron for catalysis. During...
-
recessive disorder that
causes malignant hyperphenylalaninemia due to
tetrahydrobiopterin deficiency. It is a
recessive disorder that is
accompanied by hyperphenylalaninemia...
- the
pathway that
recycles a
substance called tetrahydrobiopterin, also
known as BH4.
Tetrahydrobiopterin works with an
enzyme called phenylalanine hydroxylase...
- chemicals.
Sepiapterin can be
metabolized into
tetrahydrobiopterin via a
salvage pathway.
Tetrahydrobiopterin is an
essential cofactor in
humans for breakdown...
-
reductase deficiency (DHPRD) is a
genetic disorder affecting the
tetrahydrobiopterin (BH4)
synthesis pathway,
inherited in the
autosomal recessive pattern...
