Affiliation:
1. School of Biotechnology, Jawaharlal Nehru University, New Delhi-110067, India
2. Department of Biotechnology, Jaypee Institute of Information Technology, Sec-62, Noida, Uttar Pradesh, India
3. School of Computational and Integrative
Science, Jawaharlal Nehru University, New Delhi-110067, India
Abstract
Abstract:
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that leads to death
by progressive paralysis and respiratory failure within 2-4 years of onset. About 90-95% of ALS cases
are sporadic (sALS), and 5-10% are inherited through family (fALS). Though the mechanisms of the
disease are still poorly understood, so far, approximately 40 genes have been reported as ALS causative
genes. The mutations in some crucial genes, like SOD1, C9ORF72, FUS, and TDP-43, are majorly
associated with ALS, resulting in ROS-associated oxidative stress, excitotoxicity, protein aggregation,
altered RNA processing, axonal and vesicular trafficking dysregulation, and mitochondrial dysfunction.
Recent studies show that dysfunctional cellular pathways get restored as a result of the repair
of a single pathway in ALS. In this review article, our aim is to identify putative targets for therapeutic
development and the importance of a single suppressor to reduce multiple symptoms by focusing on
important mutations and the phenotypic suppressors of dysfunctional cellular pathways in crucial
genes as reported by other studies.
Funder
Council of Scientific and Industrial Research (CSIR), India
Publisher
Bentham Science Publishers Ltd.
Subject
Genetics (clinical),Drug Discovery,Genetics,Molecular Biology,Molecular Medicine
Cited by
3 articles.
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