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1.
Cell Rep ; 43(3): 113854, 2024 Mar 26.
Artigo em Inglês | MEDLINE | ID: mdl-38412099

RESUMO

The definition of cell metabolic profile is essential to ensure skeletal muscle fiber heterogeneity and to achieve a proper equilibrium between the self-renewal and commitment of satellite stem cells. Heme sustains several biological functions, including processes profoundly implicated with cell metabolism. The skeletal muscle is a significant heme-producing body compartment, but the consequences of impaired heme homeostasis on this tissue have been poorly investigated. Here, we generate a skeletal-muscle-specific feline leukemia virus subgroup C receptor 1a (FLVCR1a) knockout mouse model and show that, by sustaining heme synthesis, FLVCR1a contributes to determine the energy phenotype in skeletal muscle cells and to modulate satellite cell differentiation and muscle regeneration.


Assuntos
Proteínas de Membrana Transportadoras , Células Satélites de Músculo Esquelético , Camundongos , Animais , Proteínas de Membrana Transportadoras/metabolismo , Heme/metabolismo , Camundongos Knockout , Músculo Esquelético/metabolismo , Metabolismo Energético , Células Satélites de Músculo Esquelético/metabolismo , Diferenciação Celular/fisiologia
2.
Brain ; 146(6): 2227-2240, 2023 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-36729638

RESUMO

Neurodegenerative diseases are a major global health burden particularly with the increasing ageing population. Hereditary predisposition and environmental risk factors contribute to the heterogeneity of existing pathological phenotypes. Traditional clinical interventions focused on the use of small drugs have often led to failures due to the difficulties in crossing the blood-brain barrier and reaching the brain. In this regard, nanosystems can specifically deliver drugs and improve their bioavailability, overcoming some of the major challenges in neurodegenerative disease treatment. This review focuses on the use of nanosystems as an encouraging therapeutic approach targeting molecular pathways involved in localized and systematic neurodegenerative diseases. Among the latter, Friedreich's ataxia is an untreatable complex multisystemic disorder and the most widespread type of ataxia; it represents a test case to validate the clinical potential of therapeutic strategies based on nanoparticles with pleiotropic effects.


Assuntos
Ataxia de Friedreich , Doenças Neurodegenerativas , Humanos , Doenças Neurodegenerativas/tratamento farmacológico , Ataxia de Friedreich/tratamento farmacológico , Fenótipo
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