Abstract |
We studied the mitochondrial cytochrome P-450-linked monooxygenase system in livers of two patients with cerebrotendinous xanthomatosis (CTX). The three components of this system, which catalyzes steroid 27-hydroxylation, NADPH- hepatoredoxin reductase, hepatoredoxin, and cytochrome P-450s27, were stained on a nitrocellulose sheet with antibodies against NADPH- adrenodoxin reductase, adrenodoxin, and cytochrome P-450scc, respectively, from bovine adrenocortical mitochondria. The concentrations of hepatoredoxin in the patients were not significantly different from a control, but the level of NADPH- hepatoredoxin reductase was three times that of the control. Cytochrome P-450s27 was not detected in the patients, but it was present (22.8 pmol/mg of protein) in the control liver. This implies that a defect of mitochondrial cytochrome P-450s27 prevents steroid 27-hydroxylation of hepatic mitochondria in patients with CTX.
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Authors | H Miki, H Takeuchi, A Yamada, M Nishioka, Y Matsuzawa, I Hamamoto, A Hiwatashi, Y Ichikawa |
Journal | Clinica chimica acta; international journal of clinical chemistry
(Clin Chim Acta)
Vol. 160
Issue 3
Pg. 255-63
(Nov 15 1986)
ISSN: 0009-8981 [Print] Netherlands |
PMID | 3791635
(Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Ferredoxins
- Isoenzymes
- hepatoredoxin
- Cytochrome P-450 Enzyme System
- Oxygenases
- Steroid Hydroxylases
- CYP27A1 protein, human
- Cholestanetriol 26-Monooxygenase
- Ferredoxin-NADP Reductase
- NADH, NADPH Oxidoreductases
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Topics |
- Adrenal Cortex
(enzymology, ultrastructure)
- Adult
- Animals
- Brain Diseases
(complications, enzymology)
- Cattle
- Cholestanetriol 26-Monooxygenase
- Cytochrome P-450 Enzyme System
(analysis, metabolism)
- Female
- Ferredoxin-NADP Reductase
(analysis)
- Ferredoxins
(analysis)
- Humans
- Isoenzymes
(analysis)
- Mitochondria, Liver
(enzymology)
- NADH, NADPH Oxidoreductases
(analysis)
- Oxygenases
(analysis)
- Steroid Hydroxylases
(metabolism)
- Tendons
- Xanthomatosis
(complications, enzymology)
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