Ontology highlight
ABSTRACT:
SUBMITTER: Lobsiger CS
PROVIDER: S-EPMC2657393 | biostudies-literature | 2009 Mar
REPOSITORIES: biostudies-literature
Lobsiger Christian S CS Boillee Severine S McAlonis-Downes Melissa M Khan Amir M AM Feltri M Laura ML Yamanaka Koji K Cleveland Don W DW
Proceedings of the National Academy of Sciences of the United States of America 20090227 11
Neurodegeneration in an inherited form of ALS is non-cell-autonomous, with ALS-causing mutant SOD1 damage developed within multiple cell types. Selective inactivation within motor neurons of an ubiquitously expressed mutant SOD1 gene has demonstrated that mutant damage within motor neurons is a determinant of disease initiation, whereas mutant synthesis within neighboring astrocytes or microglia accelerates disease progression. We now report the surprising finding that diminished synthesis (by 7 ...[more]