Article
Epitope-tagged P(0) glycoprotein causes Charcot-Marie-Tooth-like neuropathy in transgenic mice.
The Journal of cell biology - 27 Nov 2000
Previtali S C, Quattrini A, Fasolini M, Panzeri M C, Villa A, Filbin M T, Li W, Chiu S Y, Messing A, Wrabetz L, Feltri M L
Abstract excerpt
In peripheral nerve myelin, the intraperiod line results from compaction of the extracellular space due to homophilic adhesion between extracellular domains (ECD) of the protein zero (P(0)) glycoprotein. Point mutations in this region of P(0) cause human hereditary demyelinating neuropathies such as Charcot-Marie-Tooth. We describe transgenic mice expressing a full-length P(0) modified in the ECD with a myc...
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