GCH-I Antibody (C-4): sc-271482

---

GTP cyclohydrolase I (GCH-I), a homodecamer, catalyzes the conversion of GTP into dihydroneopterin triphosphate and folate. GCH-I is the the first and rate limiting enzyme in tetrahydrobiopterin (BH4) biosynthesis. BH4 is the cofactor for tyrosine hydroxylase, a rate-limiting enzyme for dopamine synthesis, and tryptophan hydroxylase, the rate-limiting enzyme for serotonin biosynthesis. Dopamine and serotonin are neurotransmitters involved in depression, which may be associated with a deficiency of BH4. Mutations in the gene encoding GCH-I cause malignant hyperphenylalaninemia, a genetic neurological disorder characterized by abnormally high levels of serum phenylalanine, and dopa-responsive dystonia (DRD), a group of movement disorders characterized by a progressive difficulty in walking which respond to L-dopa.