title: Selective pattern of motor system damage in gamma-synuclein transgenic mice mirrors the respective pathology in amyotrophic lateral sclerosis.
abstract: Amyotrophic lateral sclerosis (ALS) is characterised by substantial loss of both upper and lower motor neuron function, with sensory and cognitive systems less affected. Though heritable forms of the disease have been described, the vast majority of cases are sporadic with poorly defined underlying pathogenic mechanisms. Here we demonstrate that the neurological pathology induced in transgenic mice by overexpression of γ-synuclein, a protein not previously associated with ALS, recapitulates key features of the disease, namely selective damage and loss of discrete populations of upper and lower motor neurons and their axons, contrasted by limited effects upon the sensory system.
authors: School of Biosciences, Cardiff University, Museum Avenue, Cardiff, CF10 3AX, UK.
authors: Owen M
authors: OM
authors: Peters
authors: Steven
authors: S
authors: Millership
authors: Tatyana A
authors: TA
authors: Shelkovnikova
authors: Ileana
authors: I
authors: Soto
authors: Lora
authors: L
authors: Keeling
authors: Anthony
authors: A
authors: Hann
authors: Nicholas
authors: N
authors: Marsh-Armstrong
authors: Vladimir L
authors: VL
authors: Buchman
authors: Natalia
authors: N
authors: Ninkina
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D000818:Animals; D001369:Axons / Q000473:pathology*; D004195:Disease Models, Animal; D051379:Mice; D008822:Mice, Transgenic; D009046:Motor Neurons / Q000378:metabolism / Q000473:pathology*; D013116:Spinal Cord / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D055698:Touch Perception / Q000502:physiology; D051845:gamma-Synuclein / Q000235:genetics* / Q000378:metabolism
chemical_list: D051845:gamma-Synuclein