Journal article

Amyotrophic Lateral Sclerosis and Autophagy: Dysfunction and Therapeutic Targeting

Azin Amin, Nirma D Perera, Philip M Beart, Bradley J Turner, Fazel Shabanpoor

CELLS | MDPI | Published : 2020

Abstract

Over the past 20 years, there has been a drastically increased understanding of the genetic basis of Amyotrophic Lateral Sclerosis. Despite the identification of more than 40 different ALS-causing mutations, the accumulation of neurotoxic misfolded proteins, inclusions, and aggregates within motor neurons is the main pathological hallmark in all cases of ALS. These protein aggregates are proposed to disrupt cellular processes and ultimately result in neurodegeneration. One of the main reasons implicated in the accumulation of protein aggregates may be defective autophagy, a highly conserved intracellular "clearance" system delivering misfolded proteins, aggregates, and damaged organelles to ..

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Grants

Awarded by National Health and Medical Research Council


Awarded by Bethlehem Griffith Research Foundation scholarship


Awarded by Motor Neurone Disease Research Institute of Australia Fellowship


Awarded by FightMND Mid-career Fellowship


Awarded by NHMRC-ARC Dementia Research Leadership Fellowship


Funding Acknowledgements

This work was supported by National Health and Medical Research Council (project grants APP1138033 to F.S. and B.J.T. and APP1104299 to B.J.T.) and Stafford Fox Medical Research Foundation (2020). A.A. is a recipient of the Bethlehem Gri ffith Research Foundation scholarship (1808). N.D.P. is a recipient of the Motor Neurone Disease Research Institute of Australia Fellowship (1810). F.S. is a recipient of the FightMND Mid-career Fellowship (04MCR). B.J.T. is a recipient of the NHMRC-ARC Dementia Research Leadership Fellowship (1137024).