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Implications of Selective Autophagy Dysfunction for ALS Pathology

1
Center for Integrative Biology, Faculty of Science, Universidad Mayor, Camino la Piramide 5750, Huechuraba 8580745, Santiago, Chile
2
Center for Genomics and Bioinformatics, Faculty of Science, Universidad Mayor, Camino la Piramide 5750, Huechuraba 8580745, Santiago, Chile
3
Escuela de Biotecnología, Facultad de Ciencias, Universidad Mayor, Camino la Piramide 5750, Huechuraba 8580745, Santiago, Chile
*
Authors to whom correspondence should be addressed.
Cells 2020, 9(2), 381; https://doi.org/10.3390/cells9020381
Received: 13 January 2020 / Revised: 1 February 2020 / Accepted: 3 February 2020 / Published: 7 February 2020
(This article belongs to the Special Issue Advances in Selective Autophagy)
Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disorder that progressively affects motor neurons in the brain and spinal cord. Due to the biological complexity of the disease, its etiology remains unknown. Several cellular mechanisms involved in the neurodegenerative process in ALS have been found, including the loss of RNA and protein homeostasis, as well as mitochondrial dysfunction. Insoluble protein aggregates, damaged mitochondria, and stress granules, which contain RNA and protein components, are recognized and degraded by the autophagy machinery in a process known as selective autophagy. Autophagy is a highly dynamic process whose dysregulation has now been associated with neurodegenerative diseases, including ALS, by numerous studies. In ALS, the autophagy process has been found deregulated in both familial and sporadic cases of the disease. Likewise, mutations in genes coding for proteins involved in the autophagy machinery have been reported in ALS patients, including selective autophagy receptors. In this review, we focus on the role of selective autophagy in ALS pathology.
Keywords: autophagy; amyotrophic lateral sclerosis; p62; TBK1; ubiquilin2; optineurin; NBR1 autophagy; amyotrophic lateral sclerosis; p62; TBK1; ubiquilin2; optineurin; NBR1
MDPI and ACS Style

Vicencio, E.; Beltrán, S.; Labrador, L.; Manque, P.; Nassif, M.; Woehlbier, U. Implications of Selective Autophagy Dysfunction for ALS Pathology. Cells 2020, 9, 381.

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