Neuronal-Specific Overexpression of a Mutant Valosin-Containing Protein Associated with IBMPFD Promotes Aberrant Ubiquitin and TDP-43 Accumulation and Cognitive Dysfunction in Transgenic Mice

Mutations in valosin-containing protein (VCP) cause a rare, autosomal dominant disease called inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia (IBMPFD). One-third of patients with IBMPFD develop frontotemporal dementia, characterized by an extensive neurodege...

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Main Authors: Rodriguez-Ortiz, Carlos J., Hoshino, Hitomi, Cheng, David, Liu-Yescevitz, Liqun, Blurton-Jones, Mathew, Wolozin, Benjamin, LaFerla, Frank M., Kitazawa, Masashi
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Investigative Pathology 2013
Assuntos:
Regular Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3730785/
https://ncbi.nlm.nih.gov/pubmed/23747512
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ajpath.2013.04.014
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