Abstract
Localized protein expression is crucial for the health and survival of axons and dendrites in a rapidly changing environment. This process, however, cannot take place without the precise spatiotemporal localization of the cellular translational machinery and of mRNA. mRNA transport and localization requires a variety of RNA-binding proteins. Here, we highlight a recent publication which presents evidence for the altered localization of the dsRNA-binding protein Staufen1 as a result of Amyotrophic Lateral Sclerosis (ALS) linked mutations, supporting the perception of ALS as a RNA spatiotemporal mislocalization disease.
Highlights
A recent publication revealed a possible role for the RNA-binding protein Staufen1 in Amyotrophic Lateral Sclerosis (ALS), demonstrating both in vitro and in vivo alterations in its localization to the synapse [1]
Alterations in RNA localization and local synthesis have been associated with neurodegenerative diseases such as Amyotrophic Lateral Sclerosis (ALS), Spinal Muscular Atrophy (SMA), Huntington’s disease (HD) and others [3, 4]
Research over the past several years has pointed to a growing role for RNA metabolism and intracellular transport in ALS, with the association of RNA binding proteins TAR DNA binding protein (TDP-43) and Fused in sarcoma/ translocated in sarcoma (FUS/TLS), whose cellular pathology include aggregation into cytoplasmic inclusions [5] with the disease
Summary
Alterations in RNA localization and local synthesis have been associated with neurodegenerative diseases such as Amyotrophic Lateral Sclerosis (ALS), Spinal Muscular Atrophy (SMA), Huntington’s disease (HD) and others [3, 4]. A recent publication revealed a possible role for the RNA-binding protein Staufen1 in Amyotrophic Lateral Sclerosis (ALS), demonstrating both in vitro and in vivo alterations in its localization to the synapse [1].
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