It has been suggested that a form of inherited dystonia responsive to levodopa might be due to an abnormality of tyrosine hydroxylase gene. This hypothesis has been tested using a cDNA tyrosine hydroxylase gene probe in three families with this disorder. No evidence for genetic linkage between the disease and tyrosine hydroxylase loci was found; it is possible that the disorder results from a p...

We have found that the induction of phenylalanine hydroxylase by hydrocortisone and serum in confluent cultures of H4-II-E-C3 rat hepatoma cells is accompanied by an increase in polysomal mRNA specific for phenylalanine hydroxylase, as measured by translation in a cell-free protein-synthesizing system. Thus, the induction is mediated largely, if not entirely, by a pretranslational mechanism, po...

The Diiron Center in Methane Monooxygenase. The dinuclear iron center of the hydroxylase component of soluble methane monooxygenase (MMO) from Methylococcus capsulatus (Bath) and Methylosinus trichosporium (OB3b) has been studied by X-ray absorption spectroscopy. Analysis of the Fe K-edge EXAFS revealed that the first shell coordination of the Fe(III)Fe(III) oxidized state of the hydroxylase fr...

A secretion from cultured bovine chromaffin cells was stimulated to examine the pattern of exocytotic fusion on the plasma membrane. Confocal microscopy revealed that dopamine-beta-hydroxylase immunofluorescence in intact cells stimulated for 20s with the nicotinic agonist 1,1-dimethyl-4-phenylpiperazinium was almost entirely punctate and evenly distributed on the cell surface. The basis for th...

Neonatal rat lung prolyl hydroxylase was synthesized in a cell-free system. Total polysomes were isolated from neonatal rat lung homogenates after Triton X-100 and deoxycholate solubilization. The polysomes were then translated in a wheat germ lysate in the presence of tritiated leucine. The radioactive product was precipitated with antibody to rat prolyl hydroxylase and co-precipitated with pr...

Kinetic, spectroscopic, and chemical evidence for the formation of specific catalytically essential complexes between the three protein components of the soluble form of methane monooxygenase from Methylosinus trichosporiurn OB3b is reported. The effects of the concentrations of the reductase and component B on the hydroxylation activity of the reconstituted enzyme system has been numerically s...

Numerous experimental data have shown that the rate of 5-hydroxytryptamine (5-HT) synthesis is enhanced by neuronal activity in mammalian central 5-HT neurons (Hamon and Glowinski, 1974; Knapp et al., 1975; Neckers et al., 1977; Boadle-Biber et al., 1983). The change of 5-HT synthesis may be due to rapid and reversible modifications of the activity of tryptophan hydroxylase (EC 1.14.16.4), whic...

results out of 617 patients, 79.6% had 21-hydroxylase deficiency (21-ohd). in 21-ohd group 94.5% had classical type and 5.5% were non-classic. among the classic type 78% had salt-wasting form (sw) and 22% simple virilizing (sv). both 21-ohd-sv and sw were diagnosed more frequently in females. frequency of other types were as follow: 11-hydroxylase deficiency (11-ohd), 13.3%; 3ß-hydroxysteroid d...

Cholesterol 7a-hydroxylase, the rate-determining enzyme in the bile acid biosynthesis pathway, is regulated in a negative feedback manner by hydrophobic bile salts returning to the liver via the portal circulation. The role of cholesterol in the regulation of cholesterol 7a-hydroxylase and the interrelationship between the cholesterol and bile acid biosynthesis pathways remain controversial. Th...