AUTHOR=Haase Georg , Rabouille Catherine TITLE=Golgi Fragmentation in ALS Motor Neurons. New Mechanisms Targeting Microtubules, Tethers, and Transport Vesicles JOURNAL=Frontiers in Neuroscience VOLUME=9 YEAR=2015 URL=https://www.frontiersin.org/journals/neuroscience/articles/10.3389/fnins.2015.00448 DOI=10.3389/fnins.2015.00448 ISSN=1662-453X ABSTRACT=
Pathological alterations of the Golgi apparatus, such as its fragmentation represent an early pre-clinical feature of many neurodegenerative diseases and have been widely studied in the motor neuron disease amyotrophic lateral sclerosis (ALS). Yet, the underlying molecular mechanisms have remained cryptic. In principle, Golgi fragmentation may result from defects in three major classes of proteins: structural Golgi proteins, cytoskeletal proteins and molecular motors, as well as proteins mediating transport to and through the Golgi. Here, we present the different mechanisms that may underlie Golgi fragmentation in animal and cellular models of ALS linked to mutations in SOD1, TARDBP (TDP-43), VAPB, and C9Orf72 and we propose a novel one based on findings in progressive motor neuronopathy