GCHFR Human
Description
GCHFR is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.
Product Specs
Product Science Overview
GTP Cyclohydrolase I Feedback Regulator (GCHFR), also known as GTP Cyclohydrolase I Feedback Regulatory Protein (GFRP), is a protein encoded by the GCHFR gene in humans. This protein plays a crucial role in the regulation of GTP Cyclohydrolase I (GCH1), an enzyme involved in the biosynthesis of tetrahydrobiopterin (BH4), a cofactor essential for the production of several neurotransmitters and nitric oxide .
GCHFR is a homodimeric protein, meaning it consists of two identical subunits. It binds to GCH1 and mediates the inhibition of GCH1 by BH4. This inhibition is reversed by the amino acid L-phenylalanine . The regulatory mechanism involves the formation of inhibited or activated complexes depending on the availability of cofactor ligands, BH4 and phenylalanine, respectively .
The regulation of GCH1 by GCHFR is vital for maintaining the balance of BH4 levels in the body. BH4 is a critical cofactor for the hydroxylation of aromatic amino acids, including phenylalanine, tyrosine, and tryptophan. These reactions are essential for the synthesis of neurotransmitters such as dopamine, serotonin, and norepinephrine . Additionally, BH4 is necessary for the production of nitric oxide, a signaling molecule involved in various physiological processes, including vasodilation and immune response .
Mutations or dysregulation of the GCHFR gene can lead to disorders related to BH4 deficiency. Such conditions can result in impaired neurotransmitter synthesis and nitric oxide production, leading to various neurological and cardiovascular disorders . Understanding the role of GCHFR in these processes is crucial for developing therapeutic strategies for these conditions.
Recombinant GCHFR refers to the protein produced through recombinant DNA technology, which involves inserting the GCHFR gene into a suitable expression system, such as bacteria or yeast, to produce the protein in large quantities. This recombinant protein can be used for research purposes to study its structure, function, and regulatory mechanisms in detail .
