Abstract:
:Tetrahydrobiopterin (BH(4)) acts as a cofactor of the aromatic amino-acid hydroxylases, and its deficiency may result in hyperphenylalaninemia (HPA) and decreased production of the neurotransmitters. BH(4) is synthesized by sepiapterin reductase (SPR) from 6-pyruvoyl-tetrahydropterin (PPH(4)). A patient with SPR deficiency shows no HPA; however, an SPR knockout mouse exhibits HPA. We have reported on the SPR-unrelated novel biosynthetic pathway from PPH(4) to BH(4) (salvage pathway II) in which 3alpha-hydroxysteroid dehydrogenase type 2 and aldose reductase work in concert. In this study, we performed the expression analysis of both proteins in humans and wild-type mice. The results of expression analysis indicated that salvage pathway II worked in human liver; however, it did not act in human brain or in mouse liver and brain. For this reason, a patient with SPR deficiency may show progressive neurological deterioration without HPA, and SPR knockout mice may exhibit HPA and abnormal locomotion activity.
journal_name
J Biochemjournal_title
Journal of biochemistryauthors
Hirakawa H,Sawada H,Yamahama Y,Takikawa S,Shintaku H,Hara A,Mase K,Kondo T,Iino Tdoi
10.1093/jb/mvp042subject
Has Abstractpub_date
2009-07-01 00:00:00pages
51-60issue
1eissn
0021-924Xissn
1756-2651pii
mvp042journal_volume
146pub_type
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