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Progressive neuronal inclusion formation and axonal degeneration in CHMP2B mutant transgenic mice

24 Feb 2012

Abstract excerpt

Mutations in the charged multivesicular body protein 2B (CHMP2B) gene cause frontotemporal lobar degeneration. The mutations lead to C-terminal truncation of the CHMP2B protein. We generated Chmp2b knockout mice and transgenic mice expressing either wild-type or C-terminally truncated mutant CHMP2B. The transgenic CHMP2B mutant mice have decreased survival and show progressive neurodegenerative changes including...

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PMID: 22366797
DOI: 10.1093/brain/aws006

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