Identification and Characterization of Coenzyme Q Synthome in the Mitochondria of Mouse Cells and Tissues

Project: National Science and Technology CouncilNational Science and Technology Council Academic Grants

Project Details

Abstract

製造合成內生性CoQ10所需之PDSS和COQ蛋白的基因或粒線體DNA突變已知會造成人類CoQ10缺乏症。我們之前發現在人類癌細胞粒線體中鑑定到含PDSS2和數個COQ蛋白之複合體,因此本研究想探討非癌化小鼠細胞和正常小鼠組織之粒線體中是否有如酵母菌研究所發現的CoQ合成體存在。本研究使用已驗證能專一辨認人類PDSS2和數個COQ蛋白的抗體,探討在小鼠細胞和組織之粒線體中,Pdss和Coq蛋白是否共同存在於同一個蛋白複合體中且其穩定性受粒線體能量狀態影響,及該複合體是否與CoQ9和其他與CoQ合成無關的蛋白結合在一起。以上方向皆為新穎目標,且在未來將能應用於CoQ缺乏之相關疾病的活體研究。

Project IDs

Project ID:PC11307-1328
External Project ID:NSTC113-2320-B182-020
StatusActive
Effective start/end date01/08/2431/07/25

Keywords

  • mouse cells
  • mouse tissues
  • endogenous coenzyme Q
  • Pdss2 protein
  • Coq proteins
  • mitochondria
  • protein complex
  • two-dimensional blue-native polyacrylamide gel electrophoresis
  • native antibody-based mobility-shift assay
  • antibody
  • co-immunoprecipitation
  • mitochondrial membrane potential
  • high-performance liquid chromatography

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