A truncated Kv1.1 protein in the brain of the megencephaly mouse: expression and interaction

• Published in: BMC neuroscience Vol. 6; no. 1; p. 65
• Main Authors: Persson, Ann-Sophie, Klement, Göran, Almgren, Malin, Sahlholm, Kristoffer, Nilsson, Johanna, Petersson, Susanna, Arhem, Peter, Schalling, Martin, Lavebratt, Catharina
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 23.11.2005; BioMed Central; BMC
• Subjects: Animals; Blotting, Western - methods; Brain - abnormalities; Brain - pathology; Cell Line; Dose-Response Relationship, Radiation; Electric Stimulation - methods; Frameshift Mutation; Gene Expression Regulation - genetics; Glycosylation; Humans; Immunohistochemistry - methods; Immunoprecipitation - methods; Kv1.1 Potassium Channel - genetics; Kv1.1 Potassium Channel - immunology; Kv1.1 Potassium Channel - metabolism; Kv1.2 Potassium Channel - metabolism; Medicin och hälsovetenskap; Membrane Potentials - genetics; Membrane Potentials - physiology; Membrane Potentials - radiation effects; Mice; Mice, Inbred BALB C; Mice, Knockout; Mice, Mutant Strains; Oocytes - physiology; Patch-Clamp Techniques - methods; Peptide Fragments - immunology; Peptide Fragments - metabolism; Protein Structure, Tertiary - genetics; Protein Transport - genetics; Transfection - methods; Xenopus

The megencephaly mouse, mceph/mceph, is epileptic and displays a dramatically increased brain volume and neuronal count. The responsible mutation was recently revealed to be an eleven base pair deletion, leading to a frame shift, in the gene encoding the potassium channel Kv1.1. The predicted MCEPH...