Journal
CELL
Volume 134, Issue 1, Pages 112-123Publisher
CELL PRESS
DOI: 10.1016/j.cell.2008.06.016
Keywords
-
Categories
Funding
- Howard Hughes Medical Institute Funding Source: Medline
- NIDDK NIH HHS [P30 DK043351, DK43351, P30 DK057521, DK57521] Funding Source: Medline
- NIGMS NIH HHS [R01 GM077465-04, GM077465, R01 GM077465] Funding Source: Medline
Ask authors/readers for more resources
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. Wevalidate 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.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available