Congenital adrenal hyperplasia because of 21-hydroxylase deficiency is closely linked to the HLA system. The lod scores in 14 informative families are presented. Apart from linkage, the 21-hydroxylase deficiency is associated with an increase of BW47 antigen and lack of B8 antigen in patients. A family with a possible recombination between the 21-hydroxylase deficiency and the HLA complex was f...

Tyrosine hydroxylase activity of synaptosomes isolated from rat brain was examined. A modified tritium-displacement assay was used, which allowed the measurement of tyrosine hydroxylase activity without the addition of either inhibitors of the metabolism of the hydroxylated products or added exogenous cofactor. The enzyme activity was strongly inhibited by the addition of exogenous catecholamin...

We present a report on two sisters who have 17 ƒ¿-hydroxylase deficiency with hyperaldosteronism. They have hypertension and hypergonadotropic hypogonadism. The steroid profiles suggest that they have 17 ƒ¿-hydroxylase deficiency. In contrast to the classical biochemical findings in 17 a-hydroxylase deficiency, both of these patients have hyperaldosteronism. Thus this report describes a new var...

By screening of culture filtrates of fungi and streptomyces for activity in inhibit dopa decarboxylase the following isoflavone compounds were obtained: psi-tectorigenen (I), genistein (II), orobol (IV), 8-hydroxygenistein (V) and a new compound (III). III was elucidated to be 3', 4', 5, 7-tetrahydroxy-8methoxy isoflavone. Among these isoflavones, IV and III showed the strongest activity in inh...

Aquayamycin was found to be a strong inhibitor of tyrosine hydroxylase. It inhibits tyrosine hydroxylase by 50 % at 3.7x10~7M. The inhibition is noncompetitive with tyrosine. The inhibition by 4x10~7 Maquayamycin increases when the concentration of 2-amino-4-hydroxy-6,7-dimethyltetrahydropteridine is increased from 2x10~4M to 1x10~3M. The inhibition of tyrosine hydroxylase by aquayamycin is rev...

Hydroxylation of 5 beta-[7 beta-3H]cholestane-3 alpha, 7 alpha-diol was studied in mitochondrial preparations from human fetal livers. The livers were obtained at legal abortions between weeks 14 and 24. In addition to hydroxylation in the 26-position, 5 beta-cholestane-3 alpha, 7 alpha-diol was hydroxylated in the 12 alpha-position. In one experiment, mitochondrial protein was solubilized and ...