Pathology of protein synthesis and degradation systems in ALS

Protein synthesis and degradation systems in neurons are among the major subjects of study in neurobiology. These systems are believed to be the main pathways involved in ALS; however, the essential pathomechanisms that underlie this disease remain obscure. In addition to the ubiquitin-proteasomal and autophagic systems, several cytoplasmic organelles are also involved in ALS. Here, we present our data and discuss the main morphological abnormalities detected in the anterior horn cells of ALS patients.