ISSN:
1573-4927
Keywords:
tetrahydrobiopterin
;
GTP cyclohydrolase
;
mutant mice
;
model systems
;
tissue levels
Source:
Springer Online Journal Archives 1860-2000
Topics:
Biology
,
Chemistry and Pharmacology
Notes:
Abstract Tetrahydrobiopterin (BH4) levels and GTP cyclohydrolase activity (GTP-CH) were measured in tissues from mutants and controls of 24 different mouse strains to identify mutants that might be suitable models for diseases which are characterized by a deficiency of the biopterin cofactor, such as parkinsonism and atypical phenylketonuria. BH4 levels and GTP-CH activity were determined in brain, liver, and spleen obtained from 24 mutants with neurological or immunological defects. BH4 levels in brain were slightly but significantly decreased in only two mutants, spastic (spa) and jittery (ji), while GTP-CH activity in brain was not significantly lower than controls in any of the strains examined. GTP-CH levels in liver were significantly decreased in four mutant strains (jittery, ji; leaner, tg la; reeler, rl; and anorexia, anx); however, BH4 levels were significantly lower only in the mutant anorexia (anx). The most significant and widespread changes in both BH4 levels and GTP-CH activity were observed in spleen. In those mutants which were most affected, BH4 levels and GTP-CH activity were decreased 85–90%.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00499000
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