Comparative proteomic analyses of Duchenne muscular dystrophy and Becker muscular dystrophy muscles: changes contributing to preserve muscle function in Becker muscular dystrophy patients.
Daniele CapitanioManuela MoriggiEnrica TorrettaPietro BarbaciniSara De PalmaAgnese ViganòHanns LochmüllerFrancesco MuntoniAlessandra FerliniMarina MoraCecilia GelfiPublished in: Journal of cachexia, sarcopenia and muscle (2020)
The mechanosensing and metabolic disruption are central nodes of DMD/BMD phenotypes. The ECM proteome composition and the metabolic rewiring in BMD lead to preservation of energy levels supporting autophagy and cell renewal, thus promoting the retention of muscle function. Conversely, DMD patients are characterized by extracellular and cytoskeletal protein dysregulation and by metabolic restriction at the level of α-ketoglutarate leading to shortage of glutamate-derived molecules that over time triggers lipogenesis and lipotoxicity.
Keyphrases
- muscular dystrophy
- duchenne muscular dystrophy
- end stage renal disease
- newly diagnosed
- ejection fraction
- chronic kidney disease
- skeletal muscle
- prognostic factors
- single cell
- peritoneal dialysis
- lymph node
- type diabetes
- metabolic syndrome
- stem cells
- small molecule
- signaling pathway
- insulin resistance
- extracellular matrix
- high fat diet induced