title: Strategies for clinical approach to neurodegeneration in Amyotrophic lateral sclerosis.
abstract: Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and ultimately fatal neurodegenerative disorder of unknown aetiology that involves the loss of upper and lower motor neurons in the cerebral cortex, brainstem and spinal cord. Significant progress in understanding the cellular mechanisms of motor neuron degeneration in ALS has not been matched with the development of therapeutic strategies to prevent disease progression, and riluzole remains the only available therapy, with only marginal effects on disease survival. More recently alterations of mRNA processing in genetically defined forms of ALS, as those related to TDP-43 and FUS-TLS gene mutations have provided important insights into the molecular networks implicated in the disease pathogenesis. Here we review some of the recent progress in promoting therapeutic strategies for neurodegeneration.
authors.affiliation: Department of Neuroscience, Clinical Neurology, University of Pisa, Pisa, Italy.
authors.forename: Cecilia
authors.initials: C
authors.lastname: Carlesi
authors.forename: Livia
authors.initials: L
authors.lastname: Pasquali
authors.forename: Selina
authors.initials: S
authors.lastname: Piazza
authors.forename: Annalisa
authors.initials: A
authors.lastname: Lo Gerfo
authors.forename: Elena
authors.initials: E
authors.lastname: Caldarazzo Ienco
authors.forename: Rosaria
authors.initials: R
authors.lastname: Alessi
authors.forename: Francesco
authors.initials: F
authors.lastname: Fornai
authors.forename: Gabriele
authors.initials: G
authors.lastname: Siciliano
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000150:complications*; D000818:Animals; D006801:Humans; D009410:Nerve Degeneration / Q000175:diagnosis / Q000209:etiology / Q000628:therapy