Peudenier S, Deleuze J F, Pham-Dinh D, Lacroix C, Boulloche J, Landrieu P
Service de Neuropédiatrie, CHU de Bicêtre, Le Kremlin-Bicêtre, France.
J Neurol. 1993 May;240(5):291-4. doi: 10.1007/BF00838164.
An unusual form of hereditary motor and sensory neuropathy characterized by a prominent disruption of the myelin lamellae is reported. In addition to detailed morphological analysis, we investigated the protein P0, which is the major protein of peripheral myelin involved in adhesion. No major gene rearrangement and no differences in P0 protein expression were observed in the present case.
据报道,一种以髓鞘板层显著破坏为特征的遗传性运动和感觉神经病的不寻常形式。除了详细的形态学分析外,我们还研究了蛋白P0,它是参与黏附的周围髓鞘的主要蛋白。在本病例中未观察到主要基因重排,且P0蛋白表达无差异。