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Merck
  • Proteomics of Cytochrome c Oxidase-Negative versus -Positive Muscle Fiber Sections in Mitochondrial Myopathy.

Proteomics of Cytochrome c Oxidase-Negative versus -Positive Muscle Fiber Sections in Mitochondrial Myopathy.

Cell reports (2019-12-19)
Marta Murgia, Jing Tan, Philipp E Geyer, Sophia Doll, Matthias Mann, Thomas Klopstock
要旨

The mosaic distribution of cytochrome c oxidase+ (COX+) and COX- muscle fibers in mitochondrial disorders allows the sampling of fibers with compensated and decompensated mitochondrial function from the same individual. We apply laser capture microdissection to excise individual COX+ and COX- fibers from the biopsies of mitochondrial myopathy patients. Using mass spectrometry-based proteomics, we quantify >4,000 proteins per patient. While COX+ fibers show a higher expression of respiratory chain components, COX- fibers display protean adaptive responses, including upregulation of mitochondrial ribosomes, translation proteins, and chaperones. Upregulated proteins include C1QBP, required for mitoribosome formation and protein synthesis, and STOML2, which organizes cardiolipin-enriched microdomains and the assembly of respiratory supercomplexes. Factoring in fast/slow fiber type, COX- slow fibers show a compensatory upregulation of beta-oxidation, the AAA+ protease AFG3L1, and the OPA1-dependent cristae remodeling program. These findings reveal compensatory mechanisms in muscle fibers struggling with energy shortage and metabolic stress.

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製品内容

Sigma-Aldrich
トリプシン ブタ膵臓由来, Proteomics Grade, BioReagent, Dimethylated
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カタラーゼ ウシ肝臓由来, lyophilized powder, 2,000-5,000 units/mg protein
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3,3′-ジアミノベンジジン 四塩酸塩 水和物, ≥96%
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フェナジンメトスルファート
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2-クロロアセトアミド, ≥98%
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フェナジン, 98%
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コハク酸ナトリウム 二塩基性 六水和物, ReagentPlus®, ≥99%
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ニトロテトラゾリウムブルークロリド, ≥90.0% (HPLC)