TY - JOUR
T1 - Selective pattern of motor system damage in gamma-synuclein transgenic mice mirrors the respective pathology in amyotrophic lateral sclerosis
AU - Peters, Owen M.
AU - Millership, Steven
AU - Shelkovnikova, Tatyana A.
AU - Soto, Ileana
AU - Keeling, Lora
AU - Hann, Anthony
AU - Marsh-Armstrong, Nicholas
AU - Buchman, Vladimir L.
AU - Ninkina, Natalia
N1 - Funding Information:
We are grateful to Gaynor Smith, Simon Brookes and Stephen Dunnett for advice and sharing behavioural testing equipment, to Robert Santer and Guy Pitt for advice and assistance with TEM and Tibor Hortobágyi for critical reading of the manuscript and to Herman van der Putten for his role in the generation and preliminary characterisation of the Thy1mγSN mice. This work was supported by The Wellcome Trust Grant 075615/Z/04/z to VLB.
PY - 2012/10
Y1 - 2012/10
N2 - 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.
AB - 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.
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U2 - 10.1016/j.nbd.2012.06.016
DO - 10.1016/j.nbd.2012.06.016
M3 - Article
C2 - 22750530
AN - SCOPUS:84864071049
VL - 48
SP - 124
EP - 131
JO - Neurobiology of Disease
JF - Neurobiology of Disease
SN - 0969-9961
IS - 1
ER -