Title |
Autophagy Dysregulation in ALS: When Protein Aggregates Get Out of Hand
|
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Published in |
Frontiers in Molecular Neuroscience, August 2017
|
DOI | 10.3389/fnmol.2017.00263 |
Pubmed ID | |
Authors |
Nandini Ramesh, Udai Bhan Pandey |
Abstract |
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that results from the loss of upper and lower motor neurons. One of the key pathological hallmarks in diseased neurons is the mislocalization of disease-associated proteins and the formation of cytoplasmic aggregates of these proteins and their interactors due to defective protein quality control. This apparent imbalance in the cellular protein homeostasis could be a crucial factor in causing motor neuron death in the later stages of the disease in patients. Autophagy is a major protein degradation pathway that is involved in the clearance of protein aggregates and damaged organelles. Abnormalities in autophagy have been observed in numerous neurodegenerative disorders, including ALS. In this review, we discuss the contribution of autophagy dysfunction in various in vitro and in vivo models of ALS. Furthermore, we examine the crosstalk between autophagy and other cellular stresses implicated in ALS pathogenesis and the therapeutic implications of regulating autophagy in ALS. |
X Demographics
Geographical breakdown
Country | Count | As % |
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United States | 4 | 24% |
United Kingdom | 2 | 12% |
Spain | 1 | 6% |
France | 1 | 6% |
Switzerland | 1 | 6% |
Unknown | 8 | 47% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 12 | 71% |
Scientists | 5 | 29% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 299 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Ph. D. Student | 69 | 23% |
Student > Bachelor | 40 | 13% |
Researcher | 38 | 13% |
Student > Master | 38 | 13% |
Student > Doctoral Student | 20 | 7% |
Other | 25 | 8% |
Unknown | 69 | 23% |
Readers by discipline | Count | As % |
---|---|---|
Biochemistry, Genetics and Molecular Biology | 76 | 25% |
Neuroscience | 53 | 18% |
Agricultural and Biological Sciences | 43 | 14% |
Medicine and Dentistry | 20 | 7% |
Pharmacology, Toxicology and Pharmaceutical Science | 8 | 3% |
Other | 26 | 9% |
Unknown | 73 | 24% |