A new mouse model of Canavan leukodystrophy displays hearing impairment due to central nervous system dysmyelination

A new mouse model of Canavan leukodystrophy displays hearing impairment due to central nervous system dysmyelination

Canavan disease is a leukodystrophy caused by mutations in the ASPA gene. This gene encodes the enzyme that converts N-acetylaspartate into acetate and aspartic acid. In Canavan disease, spongiform encephalopathy of the brain causes progressive mental retardation, motor deficit and death. We have is...

Full description

Bibliographic Details
Main Authors: Marina R. Carpinelli, Anne K. Voss, Michael G. Manning, Ashwyn A. Perera, Anne A. Cooray, Benjamin T. Kile, Rachel A. Burt
Format: Article
Language:English
Published: The Company of Biologists 2014-06-01
Series:Disease Models & Mechanisms
Subjects:
Canavan disease
Aspa
Aspartoacylase
Leukodystrophy
ENU mutagenesis
Myelin
Online Access:http://dmm.biologists.org/content/7/6/649

Internet

http://dmm.biologists.org/content/7/6/649

Similar Items