TY - JOUR
T1 - Chapter 21 Monitoring Autophagy in Lysosomal Storage Disorders
AU - Raben, Nina
AU - Shea, Lauren
AU - Hill, Victoria
AU - Plotz, Paul
PY - 2009
Y1 - 2009
N2 - 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.
AB - 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.
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U2 - 10.1016/S0076-6879(08)04021-4
DO - 10.1016/S0076-6879(08)04021-4
M3 - Article
C2 - 19216919
AN - SCOPUS:59649104665
SN - 0076-6879
VL - 453
SP - 417
EP - 449
JO - Methods in Enzymology
JF - Methods in Enzymology
IS - C
ER -