A pathophysiological study of macular mutant mouse as a model of human Menkes kinky hair disease. II. Analysis of brain metabolism using 31P- and 1H-nuclear magnetic resonance spectroscopy.:II. Analysis of Brain Metabolism Using <SUP>31</SUP>P-and<SUP>1</
スポンサーリンク
概要
- 論文の詳細を見る
We studied the brain metabolism in macular mutant mice (Ml/y, +/y), an appropriate model of Menkes kinky hair disease, using <SUP>31</SUP>P-and <SUP>1</SUP>H-NMR spectroscopy to clarify the pathophysiological mechanism of disturbed nervous function.<BR>An analysis of in vivo <SUP>31</SUP>P-NMR spectra showed a decreased phosphocreatine (PCr)/inorganic phosphate (Pi) ratio and decreased ATP levels and decreased intracellular pH in Ml/y mice at 9 days, suggesting energy failure in the brain. This associated decline in ATP levels may reflect multiple causative factors including disturbed mitochondrial respiration and ischemia secondary to circulatory failure.<BR>Brain metabolites, including PCr, creatine, lactate and 7 amino acids were easily detectable quantitatively and qualitatively by in vitro <SUP>1</SUP>H-NMR spectrum. An elevation in lactate levels and a decline in PCr/creatine ratio in Ml/y mice at 9 days were also noted with an in vitro study, supporting the in vivo data.<BR>NMR spectroscopy is a useful and promising tool to obtain the information on brain metabolism
- 一般社団法人 日本小児神経学会の論文
一般社団法人 日本小児神経学会 | 論文
- Levetiracetamが著効したSTXBP1遺伝子変異による大田原症候群の1例
- Cyclophosphamideが著効した抗NMDA受容体抗体脳炎の8歳男児例
- 重症心身障害医療からみた小児脳死に関する私見
- 幼児期発症の難治な多発性硬化症に対しcyclophosphamideパルス療法が有効であった1例
- Duchenne型筋ジストロフィー児への病気の説明に関する調査