Potential therapeutic targets for ALS: MIR206, MIR208b and MIR499 are modulated during disease progression in the skeletal muscle of patients

Lorena Di Pietro, Wanda Lattanzi, Enzo Ricci, Mario Sabatelli, Camilla Bernardini, Mirko Baranzini, Mauro Monforte, Giorgio Tasca, Amelia Conte, Giandomenico Logroscino, Fabrizio Michetti

Risultato della ricerca: Contributo in rivistaArticolo in rivistapeer review

21 Citazioni (Scopus)

Abstract

Amyotrophic lateral sclerosis (ALS) is characterized by the progressive loss of motor neurons followed by muscle weakness, paralysis and death. The disease progression is extremely variable among patients, and reliable prognostic markers have not been identified. The aim of the study was to functionally characterize selected genes and microRNAs acting in the skeletal muscle of ALS patients, taking into account the duration and evolution of the disease, in order to obtain information regarding the muscle response to ALS progression. This prospective, longitudinal study enrolled 14 ALS patients and 24 age- A nd sex-matched healthy controls. Gene expression and histological analysis indicated an increase of MIR208B and MIR499 levels and the predominance of slow fibres, respectively, in the muscles of patients with a slower disease progression. A decreased expression of MIR206 and increased levels of HDAC4, during the progression of the disease were also observed. Taken together, our data suggest that the molecular signalling that regulates re-innervation and muscle regeneration is hampered during the progression of skeletal muscle impairment in ALS. This could provide precious hints towards defining prognostic protocols, and designing novel tailored therapeutic approaches, to improve ALS patients' care and delay disease progression.
Lingua originaleEnglish
pagine (da-a)9538-N/A
Numero di pagine11
RivistaScientific Reports
Volume7
DOI
Stato di pubblicazionePubblicato - 2017

Keywords

  • Amyotrophic Lateral Sclerosis

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