A role for chloride transport in lysosomal protein degradation

  • Wartosch L
  • Stauber T
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Abstract

Loss of the lysosomal chloride transport protein ClC-7 leads to complex phenotypes in mice and man, including osteopetrosis, accumulation of lysosomal storage material, and neurodegeneration. Using novel tissue-specific ClC-7 knockout mice, we have shown that upon loss of ClC-7, lysosomal degradation of endocytosed protein is slowed down and accumulation of autophagosomes occurs.

Author-supplied keywords

  • Autophagy
  • Chloride transport
  • ClC-7
  • Lysosomal storage disease
  • Vesicular acidification

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