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Autophagy
Article
Data sources: UnpayWall
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PubMed Central
Conference object . 2012
License: CC BY NC
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Autophagy
Article . 2012 . Peer-reviewed
Data sources: Crossref
Autophagy
Article . 2012
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Autophagy in lysosomal storage disorders

Authors: Lieberman AP; Puertollano R; Raben N; Slaugenhaupt S; Walkley SU; BALLABIO, ANDREA;

Autophagy in lysosomal storage disorders

Abstract

Lysosomes are ubiquitous intracellular organelles that have an acidic internal pH, and play crucial roles in cellular clearance. Numerous functions depend on normal lysosomes, including the turnover of cellular constituents, cholesterol homeostasis, downregulation of surface receptors, inactivation of pathogenic organisms, repair of the plasma membrane and bone remodeling. Lysosomal storage disorders (LSDs) are characterized by progressive accumulation of undigested macromolecules within the cell due to lysosomal dysfunction. As a consequence, many tissues and organ systems are affected, including brain, viscera, bone and cartilage. The progressive nature of phenotype development is one of the hallmarks of LSDs. In recent years biochemical and cell biology studies of LSDs have revealed an ample spectrum of abnormalities in a variety of cellular functions. These include defects in signaling pathways, calcium homeostasis, lipid biosynthesis and degradation and intracellular trafficking. Lysosomes also play a fundamental role in the autophagic pathway by fusing with autophagosomes and digesting their content. Considering the highly integrated function of lysosomes and autophagosomes it was reasonable to expect that lysosomal storage in LSDs would have an impact upon autophagy. The goal of this review is to provide readers with an overview of recent findings that have been obtained through analysis of the autophagic pathway in several types of LSDs, supporting the idea that LSDs could be seen primarily as "autophagy disorders."

Countries
United States, Italy
Keywords

autophagy, 570, Muscles, 610, Review, sphingolipidoses, mucopolysaccharidoses, Models, Biological, lysosomal storage disorders, Lysosomal Storage Diseases, lysosomes, glycogenosis, Autophagy, Mucolipidosis Type IV, Animals, Humans

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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
358
Top 1%
Top 1%
Top 1%
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bronze