A role for chloride transport in lysosomal protein degradation.
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.
Wartosch
Lena
L
Max-Delbrück-Centrum für Molekulare Medizin and Leibniz-Institut für Molekulare Pharmakologie, Berlin, Germany. Lena.wartosch@mdc-berlin.de
Stauber
Tobias
T
D000818:Animals; D001692:Biological Transport / Q000235:genetics / Q000502:physiology; D018118:Chloride Channels / Q000235:genetics / Q000378:metabolism / Q000502:physiology*; D002712:Chlorides / Q000378:metabolism* / Q000502:physiology; D006801:Humans; D008247:Lysosomes / Q000378:metabolism*; D051379:Mice; D018345:Mice, Knockout; D011499:Protein Processing, Post-Translational / Q000235:genetics* / Q000502:physiology
D018118:Chloride Channels; D002712:Chlorides; C490803:Clcn7 protein, mouse