title: Proteomic analysis of spinal cord of presymptomatic amyotrophic lateral sclerosis G93A SOD1 mouse.
abstract: Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease, whose primary mechanisms or causes are still not defined and for which no effective treatment is available. We have recently reported that before disease onset the level of tyrosine nitrated proteins is increased in the G93A SOD1 transgenic mouse model of ALS. In the present investigation, we carried out a proteomic analysis of spinal cord extracts from G93A SOD1 mice at the presymptomatic stage of the disease to further unravel primary events in the pathogenesis and tentatively screen for potential pharmacological targets. Using a robust two-dimensional gel electrophoresis-based proteomic approach, we detected a number of proteins differentially represented in presymptomatic mice in comparison with controls. Alterations of these proteins correlate with mitochondrial dysfunction, aggregation, and stress response. Moreover, we found a variation in the isoform pattern of cyclophilin A, a molecular chaperone that protects cells from the oxidative stress.
authors.affiliation: Dulbecco Telethon Institute, Milan, Italy.
authors.forename: Tania
authors.initials: T
authors.lastname: Massignan
authors.forename: Filippo
authors.initials: F
authors.lastname: Casoni
authors.forename: Manuela
authors.initials: M
authors.lastname: Basso
authors.forename: Paola
authors.initials: P
authors.lastname: Stefanazzi
authors.forename: Emiliano
authors.initials: E
authors.lastname: Biasini
authors.forename: Massimo
authors.initials: M
authors.lastname: Tortarolo
authors.forename: Mario
authors.initials: M
authors.lastname: Salmona
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Gianazza
authors.forename: Caterina
authors.initials: C
authors.lastname: Bendotti
authors.forename: Valentina
authors.initials: V
authors.lastname: Bonetto
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism*; D000818:Animals; D021984:Cyclophilin A / Q000235:genetics; D004195:Disease Models, Animal; D015180:Electrophoresis, Gel, Two-Dimensional; D005260:Female; D051379:Mice; D008822:Mice, Transgenic; D009419:Nerve Tissue Proteins / Q000378:metabolism; D010766:Phosphorylation; D020033:Protein Isoforms / Q000235:genetics; D040901:Proteomics; D019032:Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; D013116:Spinal Cord / Q000378:metabolism; D013482:Superoxide Dismutase / Q000235:genetics*
chemical_list: D009419:Nerve Tissue Proteins; D020033:Protein Isoforms; D013482:Superoxide Dismutase; D021984:Cyclophilin A