Article
Progranulin is neurotrophic in vivo and protects against a mutant TDP-43 induced axonopathy.
PloS one - 13 Oct 2010
Laird Angela S, Van Hoecke Annelies, De Muynck Louis, Timmers Mieke, Van den Bosch Ludo, Van Damme Philip, Robberecht Wim
Abstract excerpt
Mislocalization, aberrant processing and aggregation of TAR DNA-binding protein 43 (TDP-43) is found in the neurons affected by two related diseases, amyotrophic lateral sclerosis (ALS) and frontotemporal lobe dementia (FTLD). These TDP-43 abnormalities are seen when TDP-43 is mutated, such as in familial ALS, but also in FTLD, caused by null mutations in the progranulin gene. They are also found in many patients...
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