Monitoring autophagy in lysosomal storage disorders.

  title={Monitoring autophagy in lysosomal storage disorders.},
  author={Nina Raben and Lauren E. Shea and Victoria Hill and Paul H. Plotz},
  journal={Methods in enzymology},
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… CONTINUE READING