Neuronal cells but not muscle cells are resistant to oxidative stress mediated protein misfolding and cell death: Role of molecular chaperones
| Autor: | Asish R. Chaudhuri, Arunabh Bhattacharya, Rochelle Wei, Ryan T. Hamilton |
|---|---|
| Rok vydání: | 2014 |
| Předmět: |
Protein Folding
Programmed cell death Cell Survival Biophysics Biology medicine.disease_cause Biochemistry Cell Line Mice Heat shock protein medicine Animals Myocyte Molecular Biology Cells Cultured Heat-Shock Proteins Neurons Muscle Cells Cell Death Amyotrophic Lateral Sclerosis Skeletal muscle Cell Biology Spinal cord Muscle atrophy Cell biology Mice Inbred C57BL Oxidative Stress medicine.anatomical_structure Neuron medicine.symptom Oxidative stress |
| Zdroj: | Biochemical and Biophysical Research Communications. 446:1250-1254 |
| ISSN: | 0006-291X |
| DOI: | 10.1016/j.bbrc.2014.03.097 |
| Popis: | Our recent study in a mouse model of familial-Amyotrophic Lateral Sclerosis (f-ALS) revealed that muscle proteins are equally sensitive to misfolding as spinal cord proteins despite the presence of low mutant CuZn-superoxide dismutase, which is considered to be the key toxic element for initiation and progression of f-ALS. More importantly, we observed differential level of heat shock proteins (Hsp's) between skeletal muscle and spinal cord tissues prior to the onset and during disease progression; spinal cord maintains significantly higher level of Hsp's compared to skeletal muscle. In this study, we report two important observations; (i) muscle cells (but not neuronal cells) are extremely vulnerable to protein misfolding and cell death during challenge with oxidative stress and (ii) muscle cells fail to mount Hsp's during challenge unlike neuronal cells. These two findings can possibly explain why muscle atrophy precedes the death of motor neurons in f-ALS mice. |
| Databáze: | OpenAIRE |
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