dihydrobiopterin metabolic process
Definition
Target type: biologicalprocess
The chemical reactions and pathways involving a dihydrobiopterin, a reduced pteridine derivative related to folic acid; it acts as an electron carrier in tyrosine biosynthesis and its quinoid form is produced by oxidation of tetrahydrobiopterin in several biological hydroxylation reactions. [PMID:2557335]
Dihydrobiopterin metabolic process involves the conversion of tetrahydrobiopterin (BH4) to dihydrobiopterin (BH2) and vice versa. BH4 is an essential cofactor for a variety of enzymes, including phenylalanine hydroxylase, tyrosine hydroxylase, and tryptophan hydroxylase, which are involved in the biosynthesis of neurotransmitters like dopamine, norepinephrine, and serotonin.
The process begins with the oxidation of BH4 to BH2, catalyzed by dihydrofolate reductase (DHFR). BH2 can then be reduced back to BH4 by the enzyme sepiapterin reductase (SPR). This cycle ensures a steady supply of BH4 for the biosynthesis of neurotransmitters.
Defects in dihydrobiopterin metabolic process can lead to a variety of disorders, including phenylketonuria (PKU), hyperphenylalaninemia, and neurotransmitter deficiency. These disorders can result in developmental delays, intellectual disabilities, and other neurological problems.'
"
Proteins (1)
| Protein | Definition | Taxonomy |
|---|---|---|
| Dihydropteridine reductase | A dihydropteridine reductase that is encoded in the genome of human. [PRO:DNx, UniProtKB:P09417] | Homo sapiens (human) |
Compounds (4)
| Compound | Definition | Classes | Roles |
|---|---|---|---|
| 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine | 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine : A tetrahydropyridine that is 1,2,3,6-tetrahydropyridine substituted by a methyl group at position 1 and a phenyl group at position 4. 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine: A dopaminergic neurotoxic compound which produces irreversible clinical, chemical, and pathological alterations that mimic those found in Parkinson disease. | methylpyridines; phenylpyridine; tetrahydropyridine | neurotoxin |
| 4-phenylpyridine | phenylpyridine | ||
| 1-methyl-4-(4-chlorophenyl)-1,2,3,6-tetrahydropyridine | |||
| 4-phenyl-1,2,3,6-tetrahydropyridine | 4-phenyl-1,2,3,6-tetrahydropyridine: RN given refers to parent cpd |