Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).
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Infobox references
Chemical compound
Sepiapterin, also known as 2-amino-6-[(2S)-2-hydroxypropanoyl]-7,8-dihydro-1H-pteridin-4-one, is a member of the pteridine class of organic chemicals.
Sepiapterin can be metabolized into tetrahydrobiopterin via a salvage pathway. Tetrahydrobiopterin is an essential cofactor in humans for breakdown of phenylalanine and a catalyst of the metabolism of phenylalanine, tyrosine, and tryptophan to precursors of the neurotransmitters dopamine and serotonin.
Deficiency of tetrahydrobiopterin can cause toxic buildup of phenylalanine (phenylketonuria) as well as deficiencies of dopamine, norepinephrine, and epinephrine, leading to dystonia and other neurological illnesses. This has led to clinical study of sepiapterin in humans to treat tetrahydrobiopterin deficiency.[1]
Since atherosclerosis and other circulatory diseases associated with diabetes are also associated with tetrahydrobiopterin deficiency, animal studies of the value of sepiaterin in these vascular diseases have been done. These studies show that relaxation of the blood vessels studied was impaired after animals were given sepiapterin, even though their levels of tetrahydrobiopterin were replenished.[2]
^Smith, Neil; Longo, Nicola; Levert, Keith; Hyland, Keith; Blau, Nenad (1 April 2019). "Phase I clinical evaluation of CNSA-001 (sepiapterin), a novel pharmacological treatment for phenylketonuria and tetrahydrobiopterin deficiencies, in healthy volunteers". Molecular Genetics and Metabolism. 126 (4): 406–412. doi:10.1016/j.ymgme.2019.02.001. ISSN 1096-7192. PMID 30922814. S2CID 85564348.
^Vasquez-Vivar, Jeannette; Duquiane, Damon; Whitsett, Jennifer; Kalyanaraman, B.; Rajagopalan, Sanjay (1 October 2002). "Altered Tetrahydrobiopterin Metabolism in Atherosclerosis". Arteriosclerosis, Thrombosis, and Vascular Biology. 22 (10): 1655–1661. doi:10.1161/01.ATV.0000029122.79665.D9. PMID 12377745.
Sepiapterin reductase is an enzyme that in humans is encoded by the SPR gene. Sepiapterin reductase (7,8-dihydrobiopterin:NADP+ oxidoreductase; EC 1.1...
Sepiapterin, also known as 2-amino-6-[(2S)-2-hydroxypropanoyl]-7,8-dihydro-1H-pteridin-4-one, is a member of the pteridine class of organic chemicals...
In enzymology, a sepiapterin deaminase (EC 3.5.4.24) is an enzyme that catalyzes the chemical reaction sepiapterin + H2O ⇌ {\displaystyle \rightleftharpoons...
Sepiapterin reductase deficiency is an inherited pediatric disorder characterized by movement problems, and most commonly displayed as a pattern of involuntary...
also appears to inhibit the cystine-glutamate antiporter, as well as sepiapterin reductase. It is a codrug which is a combination of sulfapyridine and...
processor released in 2023 SPR, a human gene which codes for the enzyme sepiapterin reductase Specialist registrar (SpR), physician position in Ireland and...
cyclohydrolase I (GTPCH), 6-pyruvoyltetrahydropterin synthase (PTPS), and sepiapterin reductase (SR). BH4 can be oxidized by one or two electron reactions...
enzymatic steps and proceeds from GTP, while the salvage pathway converts sepiapterin to BH4 using dihydrofolate reductase. In addition, BH2 is recycled to...
g/mol, exact mass: 237.0862 u) may refer to: Biopterin Dyspropterin Sepiapterin This set index page lists chemical structure articles associated with...
Nagatsu T (August 1991). "Cloning and sequencing of cDNA encoding human sepiapterin reductase--an enzyme involved in tetrahydrobiopterin biosynthesis". Biochemical...
Ca2+/calmodulin-dependent protein kinase II and protein kinase C of sepiapterin reductase, the terminal enzyme in the biosynthetic pathway of tetrahydrobiopterin"...
Zimprich A, Lichtner P, Hofer A, Leitner P, et al. (July 2006). "The sepiapterin reductase gene region reveals association in the PARK3 locus: analysis...
substrate of two enzymes, 6-pyruvoyltetrahydropterin 2'-reductase and sepiapterin reductase. Dyspropterin exists in all living organisms, ranging from...