Missing in Action: Dysfunctional RNA Metabolism in Oligodendroglial Cells as a Contributor to Neurodegenerative Diseases?

摘要: The formation of myelin around axons by oligodendrocytes (OL) poses an enormous synthetic and energy challenge for the glial cell. Local translation transcripts, including mRNA essential protein Myelin Basic Protein (MBP) at site deposition has been recognised as efficient mechanism to assure proper sheath assembly. Oligodendroglial precursor cells (OPCs) form synapses with neurons may localise many additional mRNAs in a similar fashion between neurons. In some diseases which demyelination occurs, abundance OPCs is present but there failure efficiently remyelinate synthesise MBP. This compromises axonal survival function. are especially sensitive cellular stress occurring neurodegenerative diseases, can impinge on their ability translate into protein. Stress causes build up cytoplasmic granules (SG) RNAs sequestered translationally stalled until ceases. Chronic particular could convert this initially protective reaction cell damage, persistence SG lead pathological aggregate or long-term block SG-associated RNAs. recent recognition that often exhibit early white matter pathology proliferation surviving OPCs, renders study stress-associated processes relevant. Here, we discuss potential dysfunction RNA regulation such Multiple Sclerosis (MS) Vanishing disease (VWM) contributions OL Amyotrophic lateral sclerosis (ALS), Alzheimer's (AD) Fragile X syndrome (FXS).