Chapter 21 Monitoring Autophagy in Lysosomal Storage Disorders

Nina Raben, Lauren Shea, Victoria Hill, Paul Plotz

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Pages (from-to)417-449
Number of pages33
JournalMethods in Enzymology
Volume453
Issue numberC
DOIs
StatePublished - 2009
Externally publishedYes

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Autophagy
Lysosomal Storage Diseases
Lysosomes
Monitoring
Defects
Research

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology

Cite this

Chapter 21 Monitoring Autophagy in Lysosomal Storage Disorders. / Raben, Nina; Shea, Lauren; Hill, Victoria; Plotz, Paul.

In: Methods in Enzymology, Vol. 453, No. C, 2009, p. 417-449.

Research output: Contribution to journalArticle

Raben, Nina ; Shea, Lauren ; Hill, Victoria ; Plotz, Paul. / Chapter 21 Monitoring Autophagy in Lysosomal Storage Disorders. In: Methods in Enzymology. 2009 ; Vol. 453, No. C. pp. 417-449.
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