J. Narayanan1*, R. Sridevi1, V. Chitra1, V. Manimaran2, T.
Tamilanban1
1Department of Pharmacology, SRM College of Pharmacy, SRM
Institute of Science and Technology, Chennai, India
2Department of Pharmaceutics, SRM College of Pharmacy, SRM
Institute of Science and Technology, Chennai, India
*For Correspondence
narayanj@srmist.edu.in
A complicated neurodegenerative
disease, amyotrophic lateral sclerosis (ALS) mostly affects motor neurons,
resulting in a gradual weakening and atrophy of muscles. This review focuses
into the pathophysiology of amyotrophic lateral sclerosis (ALS), providing
insight on the complex genetic and molecular pathways at work and the ways in
which it overlaps with frontotemporal dementia. Oxidative stress,
excitotoxicity, mitochondrial dysfunction, disruption of axonal transport,
neuroinflammation, DNA damage, and poor protein homeostasis are key
pathological characteristic features of amyotrophic lateral sclerosis (ALS).
Important hereditary factors include mutations in genes such as SOD1, FUS, and
C9ORF72. About 15% of amyotrophic lateral sclerosis (ALS) patients also show
signs of frontotemporal dementia, in addition to the characteristic motor
symptoms and cognitive and behavioral abnormalities. The condition is
characterized by aberrant TDP-43 or FUS protein aggregates, and the C9ORF72
gene mutation is the most prevalent genetic component. Physical therapy,
dietary therapies, and respiratory support are all parts of the
multidisciplinary care that is currently available to patients in an effort to
reduce symptoms and enhance their quality of life. Gene therapies and novel
pharmacological medicines that target specific disease pathways are among the
novel treatments being researched. In order to address the complex nature of
amyotrophic lateral sclerosis (ALS) and enhance patient outcomes, the field is
shifting towards early intervention techniques and personalized medication.
Keywords
Amyotrophic Lateral Sclerosis (ALS), Frontotemporal Dementia, C9ORF72 Gene, Neuroinflammation, ALS Therapy
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