Protein-protein interaction of ALS

<p>Amyotrophic lateral sclerosis (Lou Gehrig’s disease) is a rare, fatal and rapidly progressive neurodegenerative disease. It primarily affects motor neurons and in some parts of the world it is also known as Motor Neuron Disease. It damages neurons located in the cranial nerves motor nuclei, anterior horn of the spinal cord and the front cortex. It affects adults in 4^th to 6^th decade of their lives and is fatal in 80% of patients within 2-5 years. The incidence and the mortality rate of ALS are approximately 2/100,000 people per year. There is no known etiology and treatment for this disease and 90% of disease occurs sporadically. Recently a number of mechanisms have been proposed including degeneration and death of motor neurons and replacing lost neurons with gliosis. </p> <p>Approximately 10% of cases of ALS found to be familial or inherited and associated with mutation of superoxide dismutase type 1 (mSOD-1), which leads to the formation of free radicals and leading to cell death. Although these mutations occur rarely, they have provided us with valuable information about the pathogenesis and therapeutic interventions. </p> <p>We have studied ALS using several techniques together with genomics, proteomics and metabolomics to discover the various gene mutations. Proteomics is one of the non-biased, rapidly evolving technology to uncover new biological questions, protein-protein interaction and mechanisms linking to ALS. Most of the proteomics studies are conducted on animal models aimed to focus on mechanisms of disease rather than pathways of neurodegeneration. We have seen changes in protein chaperones (Hsp70), cellular functions and oxidative responses. Studies have further proven that the development of ALS is due to the expression of mSOD-1 in astrocytes and by the unidentified factor released from these cells causing loss of motor neurons. Although the proteomics studies in human as limited, further exploratory studies with human samples will likely provide more information for therapeutic strategies to treat ALS.</p> <p> </p> <p>Reference: <a></a></p> <p><a href="https://www.uptodate.com/">https://www.uptodate.com</a></p> <p><a href="https://www.als.ca/about-als/what-is-als/">https://www.als.ca/about-als/what-is-als/</a></p> <p><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2705162/">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2705162/</a></p> <p><a href="https://www.ncbi.nlm.nih.gov/pubmed?term=25932674">https://www.ncbi.nlm.nih.gov/pubmed?term=25932674</a></p> .<div><br></div><div><br>