Loss of Central Auditory Processing in a Mouse Model of Canavan Disease
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چکیده
منابع مشابه
Loss of Central Auditory Processing in a Mouse Model of Canavan Disease
Canavan Disease (CD) is a leukodystrophy caused by homozygous null mutations in the gene encoding aspartoacylase (ASPA). ASPA-deficiency is characterized by severe psychomotor retardation, and excessive levels of the ASPA substrate N-acetylaspartate (NAA). ASPA is an oligodendrocyte marker and it is believed that CD has a central etiology. However, ASPA is also expressed by Schwann cells and AS...
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Objectives: The aim of the present study was to assess the auditory lateralization ability in children with (central) auditory processing disorder. Methods: Participants were divided in two groups: 15 children with Central Auditory Processing Disorder (8-10 years) and 80 normal children (8-11 years) from both genders with pure-tone air-conduction thresholds better than 20 dB HL bilaterally a...
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Introduction Canavan disease (CD) is an autosomal recessive leukodystrophy associated with spongy degeneration of the white matter of the brain, leading to mental retardation, megalencephaly and early death {I). Brain histology in CD shows characteristic spongy degeneration of the white matter and astrocytic swelling, while neurons are spared. Aspartoacylase (ASPA) deficiency is the basic defec...
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ژورنال
عنوان ژورنال: PLoS ONE
سال: 2014
ISSN: 1932-6203
DOI: 10.1371/journal.pone.0097374