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Mechanism Of Action Of Dihydropteridine Reductase, Gabriela Arias De La Rosa
Mechanism Of Action Of Dihydropteridine Reductase, Gabriela Arias De La Rosa
Dissertations, Theses, and Capstone Projects
Human dihydropteridine reductase is an enzyme that transfers a hydride from NADH to reduce quinonoid 7,8-dihydropterin (qBH2) to 5,6,7,8-tetrahydropterin (BH4), which is a cofactor important in the production of neurotransmitters.DHPR deficiency causes a drastic form of the neurological genetic disease phenylketonuria (PKU) that does not benefit from a phenylalanine-free diet.From site-directed mutagenesis studies, mostly on Rat DHPR, we know that certain residues are important for cofactor binding, substrate binding, and hydride transfer; however, there are still some questions about how DHPR works, particularly, because there is not a crystal structure of the tertiary complex: What is …