Neuronal cells but not muscle cells are resistant to oxidative stress mediated protein misfolding and cell death: role of molecular chaperones.
Biochem Biophys Res Commun
; 446(4): 1250-4, 2014 Apr 18.
Article
em En
| MEDLINE
| ID: mdl-24685484
ABSTRACT
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.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Dobramento de Proteína
/
Estresse Oxidativo
/
Células Musculares
/
Proteínas de Choque Térmico
/
Neurônios
Idioma:
En
Ano de publicação:
2014
Tipo de documento:
Article