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| GUIMARÃES,CARINE A.; BIELLA,MAIRIS S.; LOPES,ABIGAIL; DEROZA,PEDRO F.; OLIVEIRA,MARIANA B.; MACAN,TAMIRES P.; STRECK,EMILIO L.; FERREIRA,GUSTAVO C.; ZUGNO,ALEXANDRA I.; SCHUCK,PATRÍCIA F.. |
| Increased fructose concentrations are the biochemical hallmark of fructosemia, a group of inherited disorders on the metabolic pathway of this sugar. The main clinical findings observed in patients affected by fructosemia include neurological abnormalities with developmental delay, whose pathophysiology is still undefined. In the present work we investigated the in vitro and in vivo effects of fructose on acetylcholinesterase (AchE) activity in brain structures of developing rats. For the in vitro experiments, fructose was added at increasing concentrations to the incubation medium. It was observed that fructose provoked an inhibition of acetylcholinesterase activity in cerebral cortex of 30-day-old-rats, even at low concentrations (0.1 mM). For the in... |
| Tipo: Info:eu-repo/semantics/article |
Palavras-chave: Acetylcholinesterase; Brain; Fructosel hereditary fructose intolerance. |
| Ano: 2014 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0001-37652014000401919 |
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| DIMER,NÁDIA W.; SCHUCK,PATRÍCIA F.; STRECK,EMILIO L.; FERREIRA,GUSTAVO C.. |
| Inherited metabolic diseases are a heterogeneous group of diseases caused by a punctual defect in cell metabolism, resulting in the accumulation of toxic intermediate metabolites or in the lack of important biomolecules for adequate cell functioning. D-glyceric aciduria is an inherited disease caused by a deficiency of glycerate 2-kinase activity, whose pathophysiological mechanisms remain unknown. The main clinical and neurological symptoms seen in affected patients include progressive encephalopathy, hypotonia, psychomotor and mental retardation, microcephaly, seizures, speech delay, metabolic acidosis, and even death. In this review we shall discuss these clinical and biochemical findings, as well as diagnosis and treatment of affected patients in order... |
| Tipo: Info:eu-repo/semantics/article |
Palavras-chave: D-glycerate; D-glyceric aciduria; Glycerate kinase; Glyceric acid; Organic acidurias. |
| Ano: 2015 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0001-37652015000301409 |
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