Late onset axonal Charcot-Marie-Tooth phenotype caused by a novel myelin protein zero mutation.

Bienfait, H M E; Faber, C G; Baas, F; Gabreëls-Festen, A A W M; Koelman, J H T M; Hoogendijk, J E; Verschuuren, J J; Wokke, J H J et al. · J Neurol Neurosurg Psychiatry · 2006

other · Level V

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Abstract

A late onset axonal Charcot-Marie-Tooth phenotype is described, resulting from a novel mutation in the myelin protein zero (MPZ) gene. Comparative computer modelling of the three dimensional structure of the MPZ protein predicts that this mutation does not cause a significant structural change. The primary axonal disease process in these patients points to a function of MPZ in maintenance of the myelinated axons, apart from securing stability of the myelin layer.

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