Hyperprolinemia
Mostrando 1-6 de 6 artigos, teses e dissertações.
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1. Hyperprolinemia Type IA: Benign Metabolic Anomaly or a Trigger for Brain Dysfunction?
Abstract Objective: Hyperprolinemia type I (HPI) is a rare and inherited autosomal recessive disorder caused by proline oxidase deficiency. Hyperprolinemia type 1 is biochemically defined as high plasma proline levels without urinary ?-1-pyrroline-5-carboxylate excretion. Hyperprolinemia type 1 has been considered a benign metabolic disorder, but a relation
J. inborn errors metab. screen.. Publicado em: 16/05/2019
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2. Efeitos da administração crônica de prolina no conteúdo lipídico de estruturas cerebrais de ratos
Neste trabalho foi investigado o efeito da administração crônica de prolina sobre o conteúdo total de gangliosídios, fosfolipídios e de colesterol, assim como, sobre o perfil de gangliosídios no córtex, no hipocampo, no hipotálamo e no cerebelo de ratos. Também, foi avaliado o conteúdo e o perfil de gangliosídios nas frações solúvel e resisten
Publicado em: 2008
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3. Type II hyperprolinemia. Delta1-pyrroline-5-carboxylic acid dehydrogenase deficiency in cultured skin fibroblasts and circulating lymphocytes.
Type II hyperprolinemia is an inherited abnormality in amino acid metabolism characterized by elevated plasma proline concentrations, iminoglycinuria, and the urinary excretion of delta1-pyrroline compounds. To define the enzymologic defect of this biochemical disorder, we developed a specific, sensitive radioisotopic assay for the proline degradative enzyme
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4. Renal Tubular Transport of Proline, Hydroxyproline, and Glycine in Health and in Familial Hyperprolinemia *
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5. Genetic evidence for a common enzyme catalyzing the second step in the degradation of proline and hydroxyproline.
The initial step in the degradation pathways of proline and hydroxyproline is catalyzed by proline oxidase and hydroxyproline oxidase, yielding delta 1-pyrroline-5-carboxylate and delta 1-pyrroline-3-hydroxy-5-carboxylate, respectively. The second step is the oxidation of delta 1-pyrroline-5-carboxylate to glutamate and delta 1-pyrroline-3-hydroxy-5-carboxyl
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6. Regulation of proline oxidase activity by lactate.
We found that proline oxidase, the first enzyme of the proline degradative pathway, is inhibited by lactate. The Km of the enzyme for proline increases with increasing concentrations of lactate. Since proline can be a source for gluconeogenesis, regulation of proline degradation by lactate may serve as a mechanism for allocation of metabolic fuel sources. Th