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

• Main Authors: Marina R. Carpinelli (Author), Anne K. Voss (Author), Michael G. Manning (Author), Ashwyn A. Perera (Author), Anne A. Cooray (Author), Benjamin T. Kile (Author), Rachel A. Burt (Author)
• Format: Book
• Published: The Company of Biologists, 2014-06-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

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...