Early adaptive responses in the skeletal muscle of young mice with hereditary hemochromatosis.
Mol Biol Rep
; 50(4): 3179-3187, 2023 Apr.
Article
en En
| MEDLINE
| ID: mdl-36701040
ABSTRACT
BACKGROUND:
Hereditary hemochromatosis (HH) is characterized by iron overload that can cause multiple organ dysfunction primarily due to uncontrolled iron-mediated oxidative stress. Although HH leads to muscular weakness, disorder, and fatigue, the mechanism by which HH affects skeletal muscle physiology is largely unknown.METHODS:
Using Hfe knockout mice (6-7 months old), a well-defined mouse model of HH, we examined iron status in the skeletal muscle, as well as other organs. As mitochondria are key organelle for muscular function, this study also explored how molecular markers for mitochondrial function and related systems are regulated in the HH skeletal muscle using western blots.RESULTS:
Although iron overload was evident at the systemic level, only mild iron overload was observed in the skeletal muscle of HH. Of note, mitochondrial electron transport chain complex I was upregulated in the HH skeletal muscle, which was accompanied by enhanced autophagy. However, these molecular changes were not associated with oxidative stress, suggesting altered mitochondrial metabolism in the muscle in response to iron overload.CONCLUSIONS:
These early adaptive responses may be important for supporting mitochondrial health before fully developing skeletal muscle dysfunction in HH. More studies are needed to determine the role of autophagy in the HH-related muscle mitochondrial dysfunction.Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Sobrecarga de Hierro
/
Hemocromatosis
Límite:
Animals
Idioma:
En
Revista:
Mol Biol Rep
Año:
2023
Tipo del documento:
Article
País de afiliación:
Estados Unidos