How Inflammation Pathways Contribute to Cell Death in Neuro-Muscular Disorders.

Salucci, Sara; Bartoletti Stella, Anna; Battistelli, Michela; Burattini, Sabrina; Bavelloni, Alberto; Cocco, Lucio Ildebrando; Gobbi, Pietro; Faenza, Irene

Salucci, Sara; Bartoletti Stella, Anna; Battistelli, Michela; Burattini, Sabrina; Bavelloni, Alberto; Cocco, Lucio Ildebrando; Gobbi, Pietro; Faenza, Irene.

Afiliación

Salucci S; Department of Biomolecular Sciences (DiSB), Urbino University Carlo Bo, 61029 Urbino, Italy.
Bartoletti Stella A; Cellular Signalling Laboratory, Department of Biomedical and NeuroMotor Sciences (DIBINEM), University of Bologna, 40126 Bologna, Italy.
Battistelli M; Department of Diagnostic Experimental and Specialty Medicine (DIMES), University of Bologna, 40126 Bologna, Italy.
Burattini S; Department of Biomolecular Sciences (DiSB), Urbino University Carlo Bo, 61029 Urbino, Italy.
Bavelloni A; Department of Biomolecular Sciences (DiSB), Urbino University Carlo Bo, 61029 Urbino, Italy.
Cocco LI; Laboratory of Experimental Oncology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy.
Gobbi P; Cellular Signalling Laboratory, Department of Biomedical and NeuroMotor Sciences (DIBINEM), University of Bologna, 40126 Bologna, Italy.
Faenza I; Department of Biomolecular Sciences (DiSB), Urbino University Carlo Bo, 61029 Urbino, Italy.

Biomolecules ; 11(8)2021 07 28.

Article en En | MEDLINE | ID: mdl-34439778

RESUMEN

Neuro-muscular disorders include a variety of diseases induced by genetic mutations resulting in muscle weakness and waste, swallowing and breathing difficulties. However, muscle alterations and nerve depletions involve specific molecular and cellular mechanisms which lead to the loss of motor-nerve or skeletal-muscle function, often due to an excessive cell death. Morphological and molecular studies demonstrated that a high number of these disorders seem characterized by an upregulated apoptosis which significantly contributes to the pathology. Cell death involvement is the consequence of some cellular processes that occur during diseases, including mitochondrial dysfunction, protein aggregation, free radical generation, excitotoxicity and inflammation. The latter represents an important mediator of disease progression, which, in the central nervous system, is known as neuroinflammation, characterized by reactive microglia and astroglia, as well the infiltration of peripheral monocytes and lymphocytes. Some of the mechanisms underlying inflammation have been linked to reactive oxygen species accumulation, which trigger mitochondrial genomic and respiratory chain instability, autophagy impairment and finally neuron or muscle cell death. This review discusses the main inflammatory pathways contributing to cell death in neuro-muscular disorders by highlighting the main mechanisms, the knowledge of which appears essential in developing therapeutic strategies to prevent the consequent neuron loss and muscle wasting.

Asunto(s)

Apoptosis/genética; Neuropatía Hereditaria Motora y Sensorial/metabolismo; Enfermedad de la Neurona Motora/metabolismo; Enfermedades Musculares/metabolismo; Distrofias Musculares/metabolismo; Enfermedades de la Unión Neuromuscular/metabolismo; Astrocitos/metabolismo; Astrocitos/patología; Autofagia/genética; Citocinas/genética; Citocinas/metabolismo; Proteínas de Unión al ADN/genética; Proteínas de Unión al ADN/metabolismo; Regulación de la Expresión Génica; Neuropatía Hereditaria Motora y Sensorial/genética; Neuropatía Hereditaria Motora y Sensorial/patología; Humanos; Inflamación; Microglía/metabolismo; Microglía/patología; Mitocondrias/metabolismo; Mitocondrias/patología; Enfermedad de la Neurona Motora/genética; Enfermedad de la Neurona Motora/patología; Enfermedades Musculares/genética; Enfermedades Musculares/patología; Distrofias Musculares/genética; Distrofias Musculares/patología; FN-kappa B/genética; FN-kappa B/metabolismo; Enfermedades de la Unión Neuromuscular/genética; Enfermedades de la Unión Neuromuscular/patología; Neuronas/metabolismo; Neuronas/patología; Transducción de Señal

Palabras clave

cell death; innate immune system; motor neuron disorders; neuro-muscular diseases; neuroinflammation

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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Neuropatía Hereditaria Motora y Sensorial / Enfermedad de la Neurona Motora / Apoptosis / Enfermedades de la Unión Neuromuscular / Enfermedades Musculares / Distrofias Musculares Límite: Humans Idioma: En Revista: Biomolecules Año: 2021 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Suiza

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