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Rodent models of TDP-43 proteinopathy: investigating the mechanisms of TDP-43-mediated neurodegeneration Gendron TF; Petrucelli LJ Mol Neurosci 2011[Nov]; 45 (3): 486-99Since the identification of phosphorylated and truncated transactive response DNA-binding protein 43 (TDP-43) as a primary component of ubiquitinated inclusions in amyotrophic lateral sclerosis and frontotemporal lobar degeneration with ubiquitin-positive inclusions, much effort has been directed towards ascertaining how TDP-43 contributes to the pathogenesis of disease. As with other protein misfolding disorders, TDP-43-mediated neuronal death is likely caused by both a toxic gain and loss of TDP-43 function. Indeed, the presence of cytoplasmic TDP-43 inclusions is associated with loss of nuclear TDP-43. Moreover, post-translational modifications of TDP-43, including phosphorylation, ubiquitination, and cleavage into C-terminal fragments, may bestow toxic properties upon TDP-43 and cause TDP-43 dysfunction. However, the exact neurotoxic TDP-43 species remain unclear, as do the mechanism(s) by which they cause neurotoxicity. Additionally, given our incomplete understanding of the roles of TDP-43, both in the nucleus and the cytoplasm, it is difficult to truly appreciate the detrimental consequences of aberrant TDP-43 function. The development of TDP-43 transgenic animal models is expected to narrow these gaps in our knowledge. The aim of this review is to highlight the key findings emerging from TDP-43 transgenic animal models and the insight they provide into the mechanisms driving TDP-43-mediated neurodegeneration.|*Rodentia[MESH]|Amyotrophic Lateral Sclerosis/pathology/physiopathology[MESH]|Animals[MESH]|Animals, Genetically Modified[MESH]|Behavior, Animal/physiology[MESH]|DNA-Binding Proteins/genetics/*metabolism[MESH]|Disease Models, Animal[MESH]|Frontotemporal Dementia/pathology/physiopathology[MESH]|Humans[MESH]|Inclusion Bodies/metabolism/pathology[MESH]|Mitochondria/metabolism/ultrastructure[MESH]|Nerve Degeneration/*pathology/physiopathology[MESH]|TDP-43 Proteinopathies/*pathology/physiopathology[MESH] |
