西亚试剂：A Mitochondrial Protein Compendium Elucidates Complex I Dis

A Mitochondrial Protein Compendium Elucidates Complex I Disease Biology
David J. Pagliarini,1,2,3,9 Sarah E. Calvo,1,2,3,4,9 Betty Chang,3 Sunil A. Sheth,1,2,3,4 Scott B. Vafai,1,2 Shao-En Ong,3 Geoffrey A. Walford,1,2 Canny Sugiana,5 Avihu Boneh,5,6 William K. Chen,1,2 David E. Hill,7 Marc Vidal,7 James G. Evans,8 David R. Thorburn,5,6 Steven A. Carr,3, and Vamsi K. Mootha1,2,3,

1 Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA
2 Department of Systems Biology, Harvard Medical School, Boston, MA 02446, USA
3 Broad Institute of MIT and Harvard, Cambridge, MA 02142, USA
4 Harvard-MIT Division of Health Sciences and Technology, Cambridge, MA 02139, USA
5 Murdoch Childrens Research Institute and Department of Paediatrics, University of Melbourne, Melbourne VIC 3052, Australia
6 Genetic Health Services Victoria, Royal Children's Hospital, Melbourne VIC 3052, Australia
7 Center for Cancer Systems Biology (CCSB) and Department of Cancer Biology, Dana-Farber Cancer Institute, and Department of Genetics, Harvard Medical School, Boston MA 02115, USA
8 Whitehead MIT BioImaging Center, Cambridge, MA 02139, USA

Summary

Mitochondria are complex organelles whose dysfunction underlies a broad spectrum of human diseases. Identifying all of the proteins resident in this organelle and understanding how they integrate into pathways represent major challenges in cell biology. Toward this goal, we performed mass spectrometry, GFP tagging, and machine learning to create a mitochondrial compendium of 1098 genes and their protein expression across 14 mouse tissues. We link poorly characterized proteins in this inventory to known mitochondrial pathways by virtue of shared evolutionary history. Using this approach, we predict 19 proteins to be important for the function of complex I (CI) of the electron transport chain. We validate a subset of these predictions using RNAi, including C8orf38, which we further show harbors an inherited mutation in a lethal, infantile CI deficiency. Our results have important implications for understanding CI function and pathogenesis and, more generally, illustrate how our compendium can serve as a foundation for systematic investigations of mitochondria.

• 以上资料由西亚试剂：http://www.xiyashiji.com/ 提供此产品的详细信息如密度,含量,分子式,分子量等均可在西亚官网查询   
• 相关产品如汞乙酸汞氯化汞氧化汞碘化汞硫酸汞硝酸汞溴化汞硝酸亚汞氯化亚汞乙酸苯汞碘化汞钾硫氰酸汞氯化氨基汞三氯生三氯氧磷三氯乙烯水合氯醛三氯化磷三氯化钌三氯化钛三氯化铱三氯化铑三氯硫磷三氯乙烷三氯甲烷三氯卡班TCC1,3,5-三氯苯1,2,4-三氯苯1,2,3-三氯苯无水氯化铝三氯乙酸酐三氯乙酸钠碘甲烷二碘甲烷三碘甲烷 三氟碘甲烷硫酸二甲酯氯磺酸苯硫酚苯硫酚钠3-氨基苯硫酚2,6-二氯苯硫酚2,4-二氯苯硫酚2,5-二氯苯硫酚2-甲氧基苯硫酚2-氯乙醇 等均有销售.欢迎订购