Faculty Research 1980 - 1989
Differences in the metabolism of the aromatic amino acid hydroxylase cofactor, tetrahydrobiopterin, in mutant mice with neurological and immunological defects.
Document Type
Article
Publication Date
1986
Keywords
Animal, Biopterin: aa, me, Brain: me, GTP-Cyclohydrolase: me, Immunologic-Diseases: fg, me, Liver: me, Mice, Mice-Mutant-Strains: me, Mice-Neurologic-Mutants: me, Species-Specificity, Spleen: me, SUPPORT-U-S-GOVT-NON-P-H-S, SUPPORT-U-S-GOVT-P-H-S
First Page
657
Last Page
668
JAX Source
Biochem-Genet. 1986 Oct; 24(9-10):657-68.
Grant
AM17947, RR01183
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, tgla; 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%.
Recommended Citation
Duch DS,
Bowers SW,
Woolf JH,
Davisson MT,
Maltais LJ,
Nichol CA.
Differences in the metabolism of the aromatic amino acid hydroxylase cofactor, tetrahydrobiopterin, in mutant mice with neurological and immunological defects. Biochem-Genet. 1986 Oct; 24(9-10):657-68.