Skeletal muscle dysfunction in amyotrophic lateral sclerosis: a mitochondrial perspective and therapeutic approaches


Kubat G. B., Picone P.

Neurological Sciences, cilt.45, sa.9, ss.4121-4131, 2024 (SCI-Expanded, Scopus)

  • Yayın Türü: Makale / Derleme
  • Cilt numarası: 45 Sayı: 9
  • Basım Tarihi: 2024
  • Doi Numarası: 10.1007/s10072-024-07508-6
  • Dergi Adı: Neurological Sciences
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.4121-4131
  • Anahtar Kelimeler: Amyotrophic lateral sclerosis, Mitochondria, Skeletal muscle dysfunction, Mitochondrial transplantation
  • Sağlık Bilimleri Üniversitesi Adresli: Evet

Özet

Amyotrophic lateral sclerosis (ALS) is a progressive and fatal neuromuscular disease that results in the loss of motor neurons and severe skeletal muscle atrophy. The etiology of ALS is linked to skeletal muscle, which can activate a retrograde signaling cascade that destroys motor neurons. This is why satellite cells and mitochondria play a crucial role in the health and performance of skeletal muscles. This review presents current knowledge on the involvement of mitochondrial dysfunction, skeletal muscle atrophy, muscle satellite cells, and neuromuscular junction (NMJ) in ALS. It also discusses current therapeutic strategies, including exercise, drugs, stem cells, gene therapy, and the prospective use of mitochondrial transplantation as a viable therapeutic strategy.