538 ETHYLMALONIC-ADIPIC ACIDURIA: A NEW DEFECT OF BUTYRATE OXIDATION ASSOCIATED WITH HYPOGLYCEMIA
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چکیده
منابع مشابه
Cannabinoid receptor antagonist-induced striated muscle toxicity and ethylmalonic-adipic aciduria in beagle dogs.
Ibipinabant (IBI), a potent cannabinoid-1 receptor (CB1R) antagonist, previously in development for the treatment of obesity, causes skeletal and cardiac myopathy in beagle dogs. This toxicity was characterized by increases in muscle-derived enzyme activity in serum and microscopic striated muscle degeneration and accumulation of lipid droplets in myofibers. Additional changes in serum chemistr...
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The mechanisms underlying ethylmalonic-adipic aciduria were studied in a 5-yr-old girl. Oxidation of radioactive substrates by cultured skin fibroblasts from the proband and asymptomatic family members was also determined and compared to that by normal fibroblasts and that by cells from a patient with glutaric aciduria type II. Feeding medium-chain triglycerides promptly induced vomiting and le...
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this thesis deals with the construction of some function algebras whose corresponding semigroup compactification are universal with respect to some properies of their enveloping semigroups. the special properties are of beigan a left zero, a left simple, a group, an inflation of the right zero, and an inflation of the rectangular band.
15 صفحه اولMucosal enzyme activity for butyrate oxidation; no defect in patients with ulcerative colitis.
BACKGROUND Butyrate is an important energy source for the colon and its metabolism has been reported to be defective in ulcerative colitis. One mechanism for defective butyrate metabolism in patients with ulcerative colitis could be an enzyme deficiency in the beta-oxidation pathway of butyrate. AIMS This study was undertaken to measure the activity of each enzyme involved in the beta-oxidati...
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this condition is due to deficiency in electron transport flavoprotein or electron transport fluvoprotein dehydrogenase.the clinical presentation is characteristic of fatty acid oxidation disorders. this disorder is poor prognostic and death in infancy is common.the central nervous system involving causes neurodevelopmental delay, hypotonia, and head lag. different type of seizures such as infa...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1978
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-197804001-00543