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- W2079303441 abstract "Ubiquitin positive inclusions containing truncated, phosphorylated TAR DNA protein of 43 kDa (TDP-43) are a pathological hallmark of numerous diseases including Frontotemporal Lobar Degeneration (FTLD), Alzheimer's Disease (AD) and Amyotrophic Lateral Sclerosis (ALS). Mutations in the TARDBP gene are responsible for a subset of familial ALS cases, directly implicating TDP-43 in neurodegeneration. Currently however, there is a paucity of information regarding the biological systems affected in TDP-43 proteinopathies. TDP-43 mediates splicing and is a negative regulator of transcription, but little is known regarding substrates or the pathways ultimately influenced by this activity. Furthermore, it is unclear if TDP-43 regulates identical pathways in different regions of the CNS that display TDP-43 pathology in disparate neurodegenerative diseases such as AD and ALS. To facilitate functional studies and further delineate a role in sporadic neurodegenerative disease, we have generated transgenic mice overexpressing human wild type TDP-43 under the mouse prion promoter which develop gait abnormalities, axonal degeneration and perinuclear inclusions of mitochondria. We have used the Affymetrix mouse 430 2.0 microarray to identify differential gene expression in cortex and spinal cord RNA isolated from nontransgenic and homozygote transgenic mice (n = 3 per group). To further characterize the significance of these gene changes, GeneGo pathway analysis was employed and pathways significantly altered were verified by quantitative real time PCR. We identified a total of 1271 genes in cortex and 976 in spinal cord showing significant (P < 0.05) difference in expression between nontransgenic and homozygous mice. Pathway analysis of these genes revealed abnormal expression of pathways relating to neuronal survival and function or implicated in neurodegeneration. Furthermore, these pathways were distinct for regions expressing transgenic TDP-43 that were neuropathologically normal (cortex) and abnormal (spinal cord). qPCR analysis validated microarray gene expression changes. These data suggest that TDP-43 mediated neurodegeneration may involve specific molecular pathways that differ depending on the region affected. These studies begin to identify pathways of interest that may be important in the pathogenesis of TDP-43 proteinopathies and consequently diseases such as AD and ALS." @default.
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- W2079303441 date "2010-07-01" @default.
- W2079303441 modified "2023-09-27" @default.
- W2079303441 title "P2-224: Gene expression analysis of TDP-43 transgenic mice" @default.
- W2079303441 doi "https://doi.org/10.1016/j.jalz.2010.05.1273" @default.
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