作者: Carmen Pilla , Rui Felipe de Oliveira Cardozo , Carlos Severo Dutra-Filho , Angela Terezinha Souza Wyse , Moacir Wajner
关键词:
摘要: Maple syrup urine disease (MSUD) is a metabolic disorder biochemically characterized by the accumulation of branched-chain amino acids (BCAA) and their keto (BCKA) in blood tissues. Neurological dysfunction usually present patients, but pathophysiology brain damage still obscure. Considering that energy metabolism possibly altered MSUD, main objective this study was to determine creatine kinase activity rats subjected acute chronic administration leucine. Chronic hyperleucinemia induced subcutaneous administrations 4.8 μmol leucine/g body weight, twice day, from 6th 21st postnatal day. For hyperleucinemia, 21-day-old received three acid at 3 h interval. Twelve hours after treatment or 1 one, were killed measured. The results indicated leucine leucine-treated rats. crucial role plays homeostasis brain, if these effects also occur MSUD it possible alteration enzyme may contribute found disease.