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Capturing VCP: Another Molecular Piece in the ALS Jigsaw Puzzle

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Presentation on theme: "Capturing VCP: Another Molecular Piece in the ALS Jigsaw Puzzle"— Presentation transcript:

1 Capturing VCP: Another Molecular Piece in the ALS Jigsaw Puzzle
Christopher E. Shaw  Neuron  Volume 68, Issue 5, Pages (December 2010) DOI: /j.neuron Copyright © 2010 Elsevier Inc. Terms and Conditions

2 Figure 1 Ubiquitinated TDP-43 Inclusions in the Neuronal Cytoplasm and Nucleus Are the Pathological Hallmark of ALS and FTLD-U Defective clearance of TDP-43 aggregates can arise due to mutation in three genes: TARDBP mutation may increase cytoplasmic levels of TDP-43 and promote aggregation, PGRN mutations inhibit maturation of the autophagosome, while VCP mutations inhibit TDP-43 degradation by the autophagy and proteasomal pathways. The common pathology and clinical phenotype of ALS and FTLD-U clearly implicates TDP-43 misaccumulation in their pathogenesis. Neuron  , DOI: ( /j.neuron ) Copyright © 2010 Elsevier Inc. Terms and Conditions

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