Endoplasmic reticulum stress and proteasomal system in amyotrophic lateral sclerosis.
İstanbul, Turkey. In Free Radic Biol Med, Nov 2015
Mutation of many different genes are involved in the pathogenesis such as superoxide dismutase 1, chromosome 9 open reading frame 72 and ubiquilin 2. These genes are mainly related to the antioxidant defense systems, endoplasmic reticulum stress related proteins and also protein aggregation, degradation pathways and therefore mutation of these genes cause related disorders.This review focused on the role of protein processing via endoplasmic reticulum and proteasomal system in amyotrophic lateral sclerosis which are the main players in the pathology.
Autophagy receptor defects and ALS-FTLD.
Nottingham, United Kingdom. In Mol Cell Neurosci, May 2015
Growing evidence suggests that the autophagic process can be disturbed in ALS-FTLD, including by genetic mutations affecting autophagy receptor proteins (ubiquilin-2, optineurin, SQSTM1/p62) and regulators (VCP).