Unlocking the Secrets: How ER Stress Pathway Impacts Amyotrophic Lateral Sclerosis

Published on February 15, 2023

Just like a busy highway system ensures smooth traffic flow, the endoplasmic reticulum (ER) regulates protein quality control and cellular balance. However, when misfolded proteins accumulate and disrupt this delicate system, it’s like a traffic jam that triggers ER stress. This stress signal sets off an emergency response called the unfolded protein response (UPR), which aims to restore order. Neurons, being highly sensitive, face the brunt of misfolded proteins, leading to neurodegenerative diseases like Alzheimer’s, Parkinson’s, and amyotrophic lateral sclerosis (ALS). Recent studies have discovered the intricate involvement of the ER stress pathway in ALS, shedding light on new strategies for treatment. By manipulating the UPR in experimental models of ALS, scientists have identified the critical role of ER stress in the disease’s progression. As we learn more about how ER stress contributes to ALS, exciting therapeutic avenues emerge for targeting the ER stress pathway as a potential treatment approach. To dive deeper into this intriguing research and its implications for treating ALS, delve into the full article!

The endoplasmic reticulum (ER) is a major organelle involved in protein quality control and cellular homeostasis. ER stress results from structural and functional dysfunction of the organelle, along with the accumulation of misfolded proteins and changes in calcium homeostasis, it leads to ER stress response pathway such as unfolded protein response (UPR). Neurons are particularly sensitive to the accumulation of misfolded proteins. Thus, the ER stress is involved in neurodegenerative diseases such as Alzheimer’s disease, Parkinson’s disease, prion disease and motor neuron disease (MND). Recently, the complex involvement of ER stress pathways has been demonstrated in experimental models of amyotrophic lateral sclerosis (ALS)/MND using pharmacological and genetic manipulation of the unfolded protein response (UPR), an adaptive response to ER stress. Here, we aim to provide recent evidence demonstrating that the ER stress pathway is an essential pathological mechanism of ALS. In addition, we also provide therapeutic strategies that can help treat diseases by targeting the ER stress pathway.

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