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Transgenic mouse models of CMT1A and HNPP.

Suter(U),Nave(K A) Ann N Y Acad Sci 1999-12-21

...PMP22 animal mutants with altered PMP22 gene dosage. A moderate increase in the number of PMP22 genes led to hypomyelina...

The "CMT rat": peripheral neuropathy and dysmyelination caused by transgenic ...

Niemann(S),Sereda(M W),Rossner(M),Stewart(... Ann N Y Acad Sci 1999-12-21

...PMP22). Heterozygous PMP22-transgenic rats develop muscle weakness and gait abnormalities as well as reduced nerve condu...

Nerve conduction abnormalities and neuromyotonia in genetically engineered mo...

Zielasek(J),Toyka(K V) Ann N Y Acad Sci 1999-12-21

...Pmp22 -/-, Trembler, Trembler-J, and Pmp22-overexpressing mice. This indicates abnormal impulse generation in these dysm...

Human nerve pathology caused by different mutational mechanisms of the PMP22 ...

Gabreëls-Festen(A),Wetering(R V) Ann N Y Acad Sci 1999-12-21

...PMP22 mutational mechanisms. Duplication of chromosome 17p11.2, causing an overexpression of the PMP22 gene, results in ...

Overview of Charcot-Marie-Tooth disease type 1A.

Thomas(P K) Ann N Y Acad Sci 1999-12-21

...PMP22). Inheritance is autosomal dominant in pattern. Analysis of nerve biopsies suggests that the disorder is caused by...

Overview of hereditary neuropathy with liability to pressure palsies.

Chance(P F) Ann N Y Acad Sci 1999-12-21

...PMP22 gene, are deleted in HNPP. The deletion breakpoints in HNPP map to the same intervals in which the CMT1A duplicati...

New vistas on the pathomechanism of Charcot-Marie-Tooth and related periphera...

Müller(H W) Ann N Y Acad Sci 1999-12-21

...PMP22 expression on Schwann cell growth regulation and on the capacity of genetically modified Schwann cells to myelinat...

Ultrastructural immunocytochemical abnormalities of peripheral myelin protein...

Anani(T),Sindou(P),Richard(L),Diot(M),Vall... Ann N Y Acad Sci 1999-12-21

...PMP22, P0, MBP) on sural nerve biopsy samples from 12 unrelated CMT patients. The diagnosis of CMT was based on the clin...

Distinct phenotypes associated with increasing dosage of the PLP gene: implic...

Anderson(T J),Klugmann(M),Thomson(C E),Sch... Ann N Y Acad Sci 1999-12-21

...PMP22) gene locus. Transgenic mice carrying extra copies of the wild-type Plp gene provide a valid model of PMD. Variati...

Trembler as a mouse model of CMT1A?

Garbay(B),Salles(J),Knoll(A),Boiron-Sargue... Ann N Y Acad Sci 1999-12-21

...PMP22 mRNA represent 10 and 5% of normal values in the nerves of heterozygous and homozygous Trembler, respectively. Thi...

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