Methyl oxidase

FPWSPXG Sterol 4-alpha-methyl-oxidase 1/223-2 3IArabidopsis thalianalS6% 0.2... 

Hauffa BP, Solyom J, Glaz E, Shackleton CH, Wambach G, Vecsei P, Stolecke H, Homoki J (1991) Severe hypoaldosteronism due to corticosterone methyl oxidase type II deficiency in two boys metabolic and gas chromatography-mass spectrometry studies. Eur J Pediatr 150 149-153... 

Fig. 10. Hypothesis for the interaction of the A-kinase (A-K) system activated by ACTH with the C-kinase system (C-K) in the long-term regulation of the enzymes of steroidogenesis throughout the adrenal cortex. The primary determinant of zonation of A-kinase and C-kinase activities, via zonation of cell surface receptors or other mechanisms, is hypothesized to be a gradient (e.g., of steroids) created by the pattern of blood flow in the adrenal cortex. The resultant levels of induction of steroidogenic enzymes are indicated by to show particular elevation and by to show particular lack of induction or suppression of induction. Other enzymes involved in steroidogenesis are shown in parentheses. SCC=cholesterol side-chain cleavage enzyme 3/3=3/3-hydroxysteroid dehydrogenase 17a=17a-hy-droxylase 21 =21-hydroxylase 11/3= 11/3-hydroxylase CMO= corticosterone methyl oxidase activity of 11/3-hydroxylase. Secreted steroids are indicated as B=corticosterone Aldo=aldosterone F=cortisol DHEA(S)= dehydroepiandrosterone (sulfate).

Rahier and his co-workers also characterized the activities of a sterol C-4 methyl oxidase (SMO), a 4-carboxysterol-3-hydroxysteroid dehydrogenase/ C-4 decarboxylase (3-HSD/D) and an NADPH-dependent 3-oxosteroid reductase in order to define the steps involved in C-4 demethylation in plants (Pascal et al, 1993 Rondet et al, 1999). Only recently, they have isolated two cDNAs from Arabidopsis thaliana encoding bifunctional 3-HSD/D. Transformation of a yeast ergosterol auxotroph mutant, which lacks 3-HSD/D activity, with either of these cDNAs restored ergosterol biosynthesis in the yeast mutant (Rahier et al, 2006). 

A somewhat contradictory report comes from Sprinson s group who solubilized a 4a-methyl oxidase from rat liver microsomes. Dioxygen was required as well as NAD(P)H and a cyanide-sensitive nonheme ion protein which they purified to homogeneity. A labile fraction which they could not purify was also required. Using... 

The second group of diseases includes rare defects in the final step in the biosynthesis of mineralocorticoid and glucocorticoid that lead to water-sodium disequilibrium. Adrenal corticosterone methyl oxidase II deficiency impairs the synthesis of aldosterone with consequent salt-loss in the neonatal period. Some patients, however, become completely asymptomatic later in life. Glucocorticoid suppressible hyperaldosteronism is an autosomal dominant... 

Corticosterone methyl oxidase II deficiency Adrenals 8q21-22 124080... 

Corticosterone methyl oxidase II deficiency Costeff optic atrophy syndrome Coupling state defect Creatine deficiency Creatine transporter deficiency Cu-binding P-type ATPase deficiency y-Cystathionase deficiency Cystathionine gamma-lyase deficiency Cystathionine y -synthase deficiency Cystathioninuria... 

Corticosterone methyl oxidase II deficiency 552, 569 Cortisone reductase deficiency, apparent (AERD) 553 Cramps 453... 

The biosynthesis of thebaine in P. bracteatum follows the same pathway as in the opium poppy (Hodges et al. 1977, Brochmann-Hanssen and Wunderly 1978). In contrast to earlier studies, recent isolation work has revealed thatP. bracteatum contains small amounts of codeine, neopine and oripavine as well as 14-j8-hydroxycodeinone (16) and 14-j3-hydroxycodeine (17) and both isomers of thebaine N-oxide (Kiippers et al. 1976, Phillipson et al. 1976, Theuns et al. 1977, Meshulam and Lavie 1980). It appears that the 0-methyl oxidases in P. bracteatum are extremely sluggish in comparison with the corresponding enzymes in P. somniferum. This leads to accumul-... 

Identification and characterization of 4,4-dimethyI- and 4a-methyIsterol-4a-methyl-oxidases [4,5]. 

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