A new mouse model of canavan displays hearing impairment due to central nervous system dysmyelination key words: canavan disease aspa aspartoacylase

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  • who: Benjamin T. Kile and colleagues from the Canavan disease is a leukodystrophy (disorder of the brain white matter) (Canavan, )Affected babies seem normal at birth but at about , months of age start to display hypotonia, macrocephaly, mental retardation, blindness, seizures and spasticity (Matalon et_al, ). Patients usually die at about , months of age (Toriello et_al, ). Inheritance is autosomal recessive and disease incidence is very low except for in Ashkenazi Jews, where , in, individuals carry a mutant allele (Feigenbaum et_al, have published the research: A new mouse model of Canavan displays hearing impairment due to central nervous system . . .

     

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