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Connexin 32 mutation in a Turkish family with X-linked Charcot-Marie-Tooth disease

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dc.creator TAN, MELİHA
dc.creator Sahin, Nilüfer
dc.creator KARAGÜZEL, AH
dc.creator Calapoglu, Mustafa
dc.creator KALAY, ERSAN
dc.date 2003-05-31T21:00:00Z
dc.date.accessioned 2020-10-06T11:01:28Z
dc.date.available 2020-10-06T11:01:28Z
dc.identifier b5e345d5-84fc-4aa4-ba5a-bea376a148d2
dc.identifier 10.1080/00207450390200972
dc.identifier https://avesis.sdu.edu.tr/publication/details/b5e345d5-84fc-4aa4-ba5a-bea376a148d2/oai
dc.identifier.uri http://acikerisim.sdu.edu.tr/xmlui/handle/123456789/70016
dc.description In the present work, we describe a large Turkish family (N=39) with Charcot-Marie-Tooth disease, which is the most commonly inherited peripheral neuropathy. The subjects were from four generations, including six hemizygote patients and nine heterozygote carrier females: Symptoms appeared in late childhood in males (mean age=13.5) but later in carrier females (mean age=33.5). The peripheral nerve conduction was more severely affected in males than females. Genomic DNA was isolated from peripheral white blood cells. Using SSCP technique (single strand conformation polymorphism analysis); abnormal patterns of migration were observed in 15 subjects: 6 of them were hemizygote males and 9 were heterozygote carrier females. We identified a mutation of the Cx32 gene, consisting of a guanine to adenine transition at position 271 (271 G-A). The results suggested relations between degenerative processes and position of Cx32 mutations.
dc.language eng
dc.rights info:eu-repo/semantics/closedAccess
dc.title Connexin 32 mutation in a Turkish family with X-linked Charcot-Marie-Tooth disease
dc.type info:eu-repo/semantics/article


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