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Autophagy01:27

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Autophagy is a self-digesting process by which a cell protects itself from threats both within and outside the cell, ranging from abnormal proteins to invading bacteria. In this process, obsolete components of the cell and invading microbes are degraded by hydrolytic enzymes active in an acidic environment of the lysosomal lumen.
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Christian de Duve discovered “autophagy,” a process in which cellular components are engulfed by membrane-bound organelles called autophagosomes. The autophagosomes then fuse with lysosomes to digest the enclosed contents. Autophagy is generally activated in cells to prevent cell death. However, cell death is triggered when the damage is beyond repair.
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Implications of Selective Autophagy Dysfunction for ALS Pathology.

Emiliano Vicencio1, Sebastián Beltrán1, Luis Labrador1

  • 1Center for Integrative Biology, Faculty of Science, Universidad Mayor, Camino la Piramide 5750, Huechuraba 8580745, Santiago, Chile.

Cells
|February 13, 2020
PubMed
Summary

Selective autophagy, a cellular degradation process, is implicated in Amyotrophic Lateral Sclerosis (ALS) pathology. Dysregulation of this process and mutations in autophagy-related genes are observed in ALS patients, highlighting its crucial role.

Keywords:
NBR1TBK1amyotrophic lateral sclerosisautophagyoptineurinp62ubiquilin2

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Area of Science:

  • Neuroscience
  • Cell Biology
  • Genetics

Background:

  • Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease affecting motor neurons.
  • The exact cause of ALS remains unknown, but cellular dysfunctions like loss of RNA/protein homeostasis and mitochondrial issues are involved.
  • Selective autophagy degrades cellular components like protein aggregates and damaged mitochondria, processes relevant to ALS.

Purpose of the Study:

  • To review the role of selective autophagy in the pathology of Amyotrophic Lateral Sclerosis (ALS).

Main Methods:

  • Literature review focusing on selective autophagy mechanisms and their connection to ALS.
  • Analysis of studies investigating autophagy dysregulation in familial and sporadic ALS.
  • Examination of genetic links between autophagy machinery mutations and ALS.

Main Results:

  • Autophagy, particularly selective autophagy, is frequently dysregulated in ALS.
  • Mutations in genes encoding selective autophagy receptors are found in ALS patients.
  • Impaired selective autophagy contributes to the accumulation of toxic cellular components in ALS.

Conclusions:

  • Selective autophagy plays a significant role in ALS pathogenesis.
  • Targeting selective autophagy pathways may offer therapeutic strategies for ALS.
  • Further research into selective autophagy in ALS is warranted.