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PMID: 19216919 Published · ppublish English Journal Article

Monitoring autophagy in lysosomal storage disorders.

Methods in enzymology ·Vol. 453 ·2009-00-00 ·Pages 417-49

Raben N, Shea L, Hill V, Plotz P

Abstract

Lysosomes are the final destination of the autophagic pathway. It is in the acidic milieu of the lysosomes that autophagic cargo is metabolized and recycled. One would expect that diseases with primary lysosomal defects would be among the first systems in which autophagy would be studied. In reality, this is not the case. Lysosomal storage diseases, a group of more than 60 diverse inherited disorders, have only recently become a focus of autophagic research. Studies of these clinically severe conditions promise not only to clarify pathogenic mechanisms, but also to expand our knowledge of autophagy itself. In this chapter, we will describe the lysosomal storage diseases in which autophagy has been explored, and present the approaches used to evaluate this essential cellular pathway.

MeSH Terms
Animals Autophagy/physiology Cells, Cultured Fluorescent Antibody Technique Humans Immunoblotting Lysosomal Storage Diseases/metabolism Lysosomes/metabolism Mice Microscopy, Electron Mitochondria/metabolism
Authors & Affiliations
4 authors, click to expand affiliations / ORCID
Raben Nina
The Arthritis and Rheumatism Branch, NIAMS, National Institutes of Health, Bethesda, Maryland, USA.
Shea Lauren
Hill Victoria
Plotz Paul
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Article Info
Journal
Methods in enzymology
Abbr.
Methods Enzymol
ISSN
1557-7988
DOI
10.1016/S0076-6879(08)04021-4
Published
2009-00-00
Pages
417-49
Language
English
Region
United States
NLM ID
0212271
PMCID
PMC2669416
Subset
IM
Grants
Intramural NIH HHS · Z99 AR999999 · United States
External Links
PubMed source DOI: 10.1016/S0076-6879(08)04021-4 PMC Full Text
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