Ontology highlight
ABSTRACT:
SUBMITTER: Wishart TM
PROVIDER: S-EPMC2951867 | biostudies-literature | 2010 Nov
REPOSITORIES: biostudies-literature
Wishart Thomas M TM Huang Jack P-W JP Murray Lyndsay M LM Lamont Douglas J DJ Mutsaers Chantal A CA Ross Jenny J Geldsetzer Pascal P Ansorge Olaf O Talbot Kevin K Parson Simon H SH Gillingwater Thomas H TH
Human molecular genetics 20100812 21
Reduced expression of the survival motor neuron (SMN) gene causes the childhood motor neuron disease spinal muscular atrophy (SMA). Low levels of ubiquitously expressed SMN protein result in the degeneration of lower motor neurons, but it remains unclear whether other regions of the nervous system are also affected. Here we show that reduced levels of SMN lead to impaired perinatal brain development in a mouse model of severe SMA. Regionally selective changes in brain morphology were apparent in ...[more]