A genetic model of amyotrophic lateral sclerosis in zebrafish displays phenotypic hallmarks of motoneuron disease.

ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that, for approximately 80% of patients, is fatal within five years of diagnosis. To better understand ALS, animal models have been essential; however, only rodent models of ALS exhibit the major hallmarks of the disease. Here, we report the generation of transgenic zebrafish overexpressing mutant Sod1. The construct used to generate these lines contained the zebrafish sod1 gene and approximately 16 kb of flanking sequences. We generated lines expressing the G93R mutation, as well as lines expressing wild-type Sod1. Focusing on two G93R lines, we found that they displayed the major phenotypes of ALS. Changes at the neuromuscular junction were observed at larval and adult stages. In adulthood the G93R mutants ex

SUBMITTER: Ramesh T 

PROVIDER: S-EPMC2931540 | biostudies-literature | 2010 Sep-Oct

REPOSITORIES: biostudies-literature