The molecular links between TDP‐43 dysfunction and neurodegeneration
E Buratti, FE Baralle - Advances in genetics, 2009 - Elsevier
TDP-43 nuclear protein is involved in several major neurodegenerative diseases that
include frontotemporal lobar degeneration with ubiquitin (FTLD-U) bodies and amyotrophic
lateral sclerosis (ALS). As a consequence, the role played by this protein in both normal and
diseased cellular metabolism has come under very close scrutiny. In the neuronal tissues of
affected individuals TDP-43 undergoes aberrant localization to the cytoplasm to form
insoluble aggregates. Furthermore, it is subject to degradation, ubiquitination, and …
include frontotemporal lobar degeneration with ubiquitin (FTLD-U) bodies and amyotrophic
lateral sclerosis (ALS). As a consequence, the role played by this protein in both normal and
diseased cellular metabolism has come under very close scrutiny. In the neuronal tissues of
affected individuals TDP-43 undergoes aberrant localization to the cytoplasm to form
insoluble aggregates. Furthermore, it is subject to degradation, ubiquitination, and …
