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CMT 1B

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Deb - this is from an abstract off Pub Med I posted a few weeks ago:

perhaps you might want to get a copy of the entire article - it is from

the European Journal of Neurology, Sept. 2003 ~ G

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Charcot-Marie-Tooth disease type 1B (CMT1B) is a

demyelinating neuropathy inherited as an autosomal dominant trait.

The majority of CMT1B cases are caused by mutations in the

myelin protein zero (P0) gene (MPZ). Only a few mutations in

MPZ gene have been reported to be associated with focally folded

myelin sheaths. We have studied five patients from one family with

five generations, affected by CMT1B disease. The morphological

studies of sural nerve biopsy performed in the proband revealed

fibers with focally folded myelin. DNA sequencing analysis

showed the Asn131Lys mutation in the MPZ gene in three members

of the affected family.

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