CoQ10 supplementation: a new treatment modality in steroid-resistant nephrotic syndrome with unknown molecular etiology
作者: Dincel N Ozdemir K Kara O.D Yılmaz E Gun Z.H Berdeli A Mir S.
刊名: International Journal of Medicine and Biomedical Research, 2014, Vol.3 (3)
来源数据库: Michael Joanna Publications
DOI: 10.14194/ijmbr.3.3.1
关键词: CoQ 10CoQ2 nephropathySRNSquinonechildrenmutation
原始语种摘要: Background: Steroid-resistant nephrotic syndrome (SRNS) is a common problem in paediatric nephrology practice which tends to progress to end-stage renal disease. SRNS-causing genes are expressed in the glomerular podocyte which is a specialized cell type. However, there are more than 80% of SRNS cases with unknown molecular etiology. Coenzyme Q10 (CoQ10) has an important role in the mitochondrial electron-transport chain. Synthesis of CoQ10 is a complex pathway, involving enzymes from various genomes; termed from CoQ1 to CoQ10. Due to sequenced reactions of synthesis, enzymes and regulatory proteins are needed in obligation. So any dysfunction here; affects the biosynthesis of CoQ10. Aim: We here described quinone-responsive six patients having CoQ2 mutations, presented with...
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关键词翻译
关键词翻译
  • resistant 耐久的
  • nephrotic 肾病的
  • etiology 病原学
  • steroid 甾族化合物
  • molecular 分子的
  • unknown 不知道的
  • podocyte 足状突细胞
  • renal 肾的
  • treatment 处理
  • nephrology 肾病学