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Authors :
Hiroshi Ichinose
Toshiharu Nagatsu
Source :
Cellular and Molecular Neurobiology. 19:57-66
Publication Year :
1999
Publisher :
Springer Science and Business Media LLC, 1999.

Abstract

1. Catecholamine (dopamine, norepinephrine, and epinephrine) biosynthesis is regulated by tyrosine hydroxylase (TH). TH activity is regulated by the concentration of the cofactor tetrahydrobiopterin (BH4), whose level is regulated by GTP cyclohydrolase I (GCH) activity. Thus, GCH activity indirectly regulates TH activity and catecholamine levels. 2. TH activity in the nigrostriatal dopaminergic neurons is most sensitive to the decrease in BH4. 3. Mutations of GCH result in reductions in GCH activity, BH4, TH activity, and dopamine, causing either recessively inherited GCH deficiency or dominantly inherited hereditary progressive dystonia [HPD; Segawa's disease; also called dopa-responsive dystonia (DRD)]. 4. In juvenile parkinsonism and Parkinson's disease, which have dopamine deficiency in the basal ganglia as HPD/DRD, the GCH gene may be normal, and the molecular mechanism of the dopamine deficiency in the basal ganglia is different from that in HPD/DRD.

Details

ISSN :
02724340
Volume :
19
Database :
OpenAIRE
Journal :
Cellular and Molecular Neurobiology
Accession number :
edsair.doi...........8134e8d2a54cf26d1bb28c25fdf5d41f
Full Text :
https://doi.org/10.1023/a:1006912523846