作者: Laura A. Harsan , Patrick Poulet , Blandine Guignard , Nathalie Parizel , Robert P. Skoff
DOI: 10.1002/JNR.21201
关键词: Cell biology 、 Aquaporin 4 、 Pelizaeus–Merzbacher disease 、 Neuroscience 、 Muscle hypertrophy 、 Proteolipid protein 1 、 Astrocyte 、 Oligodendrocyte 、 Genetically modified mouse 、 White matter 、 Chemistry
摘要: The effect of a proteolipid protein (PLP) mutation on the developing white matter anisotropy was examined by diffusion tensor magnetic resonance imaging (DT-MRI) in noninvasive study mouse model Pelizaeus-Merzbacher disease (PMD). jimpy PLP mice produces an irreversible dysmyelination males, whereas heterozygous females exhibit transient hypomyelination, as assessed longitudinal same during development. Modifications different individual DT-MRI parameters were highlighted specific changes tissue structures caused that includes axonal abnormalities, and recovery. Astrocytic hypertrophy is striking cellular event dysmyelinated brain, where most axons or bundles fibers are entirely wrapped astrocyte cytoplasmic processes, so its influences for first time. data brain compared with those obtained from (oligo-TTK) transgenic mice, induced oligodendrocyte killing, which have mild (Jalabi et al., 2005), recovering females, reduced hypertrophy. unique morphological feature astrocytes males coupled increase water channel aquaporin 4 (AQP4) found to facilitate directional matter. In addition major two myelin loss modifications, amplified magnitude radial axial diffusions attributed principally strongly pronounced