Methylmalonate
Mostrando 1-12 de 12 artigos, teses e dissertações.
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1. ATIVIDADE ANTICONVULSIVANTE DO ÓLEO DE PEIXE / ANTICONVULSANT ACTIVITY OF FISH OIL
Methylmalonic acidemias are inherited metabolic disorders characterized by methylmalonate (MMA) accumulation and neurological dysfunction, including seizures. Dietary fatty acids are known as an important energy source and reduce seizure activity in selected acute animal models. This study investigates whether the chronic treatment with fish oil or with olei
Publicado em: 2010
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2. PROSTAGLANDIN E2 POTENTIATES METHYLMALONATE-INDUCED SEIZURES / PROSTAGLANDINA E2 POTENCIALIZA AS CONVULSÕES INDUZIDAS POR METILMALONATO
Methylmalonic acidemias comprehend a group of innate error of the metabolism (EIM)characterized clinically and biochemically for the tissue accumulation of acid methylmalonic (MMA)and neurological dysfunction, including seizures. The clinical experience suggests that infections precipitate metabolic crises in methylmalonic acidemic patients. Since it has bee
Publicado em: 2009
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3. Disfunção mitocondrial induzida por metilmalonato e 3-nitropropionato / Mitochondrial dysfunction induced by methylmalonate and 3-nitropropionate
A acidemia metilmalônica (MMAemia) é uma desordem metabólica hereditária do metabolismo de aminoácidos com cadeia ramificada e de ácidos graxos com cadeia ímpar, envolvendo um defeito na conversão de metilmalonil-CoA a succinil-CoA. Manifestações sistêmicas e neurológicas nesta doença são relacionadas com o acúmulo de metilmalonato (MMA) em te
Publicado em: 2004
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4. Failure of the Normal Ureagenic Response to Amino Acids in Organic Acid-loaded Rats: PROPOSED MECHANISM FOR THE HYPERAMMONEMIA OF PROPIONIC AND METHYLMALONIC ACIDEMIA
Propionic and methylmalonic acidemia are both known to be associated with hyperammonemia. Rats injected with 10 or 20 mmol/kg of propionate or 20 mmol/kg of methylmalonate, along with 1.5 g/kg of a mixture of amino acids, developed severe hyperammonemia, whereas rats administered the same dosages of acetate did not. In vitro, neither propionyl nor methylmalo
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5. Vitamin B12S-promoted model rearrangement of methylmalonate to succinate is not a free radical reaction.
To probe for free radical intermediates in the model methylmalonate to succinate rearrangements promoted by vitamin B12s, a model series with a pentenyl side chain radical trap has been devised. The control free radical, generated by tri-n-butyltin hydride treatment of bromomethyl-pentenylmalonate thioester, undergoes rapid cyclization to the six-membered ri
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6. 3-Hydroxypropionate: Significance of β-Oxidation of Propionate in Patients with Propionic Acidemia and Methylmalonic Acidemia
[l-14C]Propionate administered intravenously was metabolized to methylmalonate, to 3-hydroxypropionate, and to methylcitrate in the urine of a patient with methylmalonic acidemia. L-[U-14C]Isoleucine and L-[U-14C]valine were also converted to urinary methylmalonate and to 3-hydroxypropionate in the patient. Two patients with propionic acidemia due to a defec
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7. Regulation of Valine Catabolism in Pseudomonas putida
The activities of six enzymes which take part in the oxidation of valine by Pseudomonas putida were measured under various conditions of growth. The formation of four of the six enzymes was induced by growth on d- or l-valine: d-amino acid dehydrogenase, branched-chain keto acid dehydrogenase, 3-hydroxyisobutyrate dehydrogenase, and methylmalonate semialdehy
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8. CONGENITAL METHYLMALONIC ACIDEMIA: ENZYMATIC EVIDENCE FOR TWO FORMS OF THE DISEASE*
Methylmalonic acidemia is an inherited metabolic disorder thus far found in children and characterized by the excessive excretion of methylmalonate in the urine. Typically these children exhibit vomiting, lethargy, ketoacidosis, and failure to grow. Many of the patients are mentally retarded and die early in life. Two variants of this disease are known. In o
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9. Inherited propionyl-CoA carboxylase deficiency in “ketotic hyperglycinemia”
Cultured fibroblasts from a young girl with ketotic hyperglycinemia were unable to oxidize propionate-14C to 14CO2, but oxidized methylmalonate-14C and succinate-14C normally. This block in propionate catabolism was shown to result from a lack of propionyl-CoA carboxylase activity. The carboxylase deficiency was not due to the presence of an intracellular in
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10. Ethyl-substituted erythromycin derivatives produced by directed metabolic engineering
A previously unknown chemical structure, 6-desmethyl-6-ethylerythromycin A (6-ethylErA), was produced through directed genetic manipulation of the erythromycin (Er)-producing organism Saccharopolyspora erythraea. In an attempt to replace the methyl side chain at the C-6 position of the Er polyketide backbone with an ethyl moiety, the methylmalonate-specific
The National Academy of Sciences.
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11. Carbon-13 Nuclear Magnetic Resonance Study of Metabolism of Propionate by Escherichia coli
We have evaluated the use of [1,2-13C2]propionate for the analysis of propionic acid metabolism, based on the ability to distinguish between the methylcitrate and methylmalonate pathways. Studies using propionate-adapted Escherichia coli MG1655 cells were performed. Preservation of the 13C-13C-12C carbon skeleton in labeled alanine and alanine-containing pep
American Society for Microbiology.
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12. Origins of the 2,4-Dinitrotoluene Pathway
The degradation of synthetic compounds requires bacteria to recruit and adapt enzymes from pathways for naturally occurring compounds. Previous work defined the steps in 2,4-dinitrotoluene (2,4-DNT) metabolism through the ring fission reaction. The results presented here characterize subsequent steps in the pathway that yield the central metabolic intermedia
American Society for Microbiology.