sepiapterin reductase


sepiapterin reductase
se·pi·ap·ter·in re·duc·tase (se″pe-apґtər-in re-dukґtās) [EC 1.1.1.153] an enzyme of the oxidoreductase class that catalyzes the final two-step, NADPH-dependent, reduction reaction in the biosynthesis of tetrahydrobiopterin (BH4), acting on the 6-pyruvoyltetrahydropterin intermediate formed by 6-pyruvoyltetrahydropterin synthase. It also catalyzes a step in the alternative salvage pathway of BH4 synthesis, and the reduction of various other carbonyl substances. Deficiency of the enzyme, an autosomal recessive trait, leads to BH4 and neurotransmitter deficiencies without hyperphenylalaninemia, manifesting as dopa-responsive dystonia.

Medical dictionary. 2011.

Look at other dictionaries:

  • Sepiapterin reductase — In enzymology, a sepiapterin reductase (EC number|1.1.1.153) is an enzyme that catalyzes the chemical reaction:7,8 dihydrobiopterin + NADP+ ightleftharpoons sepiapterin + NADPH + H+Thus, the two substrates of this enzyme are 7,8 dihydrobiopterin… …   Wikipedia

  • Sepiapterin-Reduktase — Bändermodell des SPR Dimers des Menschen nach …   Deutsch Wikipedia

  • Sepiapterin — Strukturformel Allgemeines Name Sepiapterin Andere Namen …   Deutsch Wikipedia

  • Dihydrofolate reductase — Ribbon diagram of human dihydrofolate reductase in complex with folate (blue). From PDB 1DRF …   Wikipedia

  • Methylenetetrahydrofolate reductase — methylene tetrahydrofolate reductase [NAD(P)H] Ribbon diagram of the active site of E. coli MTHFR. The flavin cofactor (top) is shown interacting with the bound substrate NADH.[1] …   Wikipedia

  • Dihydrobiopterin reductase — Dihydrobiopterin is a compound produced in the synthesis of dopamine, norepinephrine and epinephrine through production of the intermediate 3,4 dihydroxy phenylalanine, also known as dopa. Phenylalanine is converted into dopa using… …   Wikipedia

  • SPR (gene) — Sepiapterin reductase (7,8 dihydrobiopterin:NADP+ oxidoreductase), also known as SPR, is a human gene.cite web | title = Entrez Gene: SPR sepiapterin reductase (7,8 dihydrobiopterin:NADP+ oxidoreductase)| url =… …   Wikipedia

  • List of EC numbers (EC 1) — This list contains a list of EC numbers for the first group, EC 1, oxidoreducatases, placed in numerical order as determined by the Nomenclature Committee of the International Union of Biochemistry and Molecular Biology.EC 1.1 Acting on the CH OH …   Wikipedia

  • Metabolism — Cell metabolism redirects here. For the journal, see Cell Metabolism. Structure of adenosine triphosphate, a central intermediate in energy metabolism Metabolism (from Greek: μεταβολή metabolē , change or Greek: μεταβολισμός metabolismos,… …   Wikipedia

  • QDPR — (quinoid dihydropteridine reductase) is a human gene that produces the enzyme quinoid dihydropteridine reductase. This enzyme is part of the pathway that recycles a substance called tetrahydrobiopterin, also known as BH4. Tetrahydrobiopterin… …   Wikipedia

Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”

We are using cookies for the best presentation of our site. Continuing to use this site, you agree with this.