[HTML][HTML] Targeted depletion of TDP-43 expression in the spinal cord motor neurons leads to the development of amyotrophic lateral sclerosis-like phenotypes in mice

LS Wu, WC Cheng, CKJ Shen - Journal of Biological Chemistry, 2012 - ASBMB
ALS, or amyotrophic lateral sclerosis, is a progressive and fatal motor neuron disease with
no effective medicine. Importantly, the majority of the ALS cases are with TDP-43
proteinopathies characterized with TDP-43-positive, ubiquitin-positive inclusions (UBIs) in
the cytosol. However, the role of the mismetabolism of TDP-43 in the pathogenesis of ALS
with TDP-43 proteinopathies is unclear. Using the conditional mouse gene targeting
approach, we show that mice with inactivation of the Tardbp gene in the spinal cord motor …