Lysosomal degradation of endocytosed proteins depends on the chloride transport protein ClC-7.

Abstract

Mutations in either ClC-7, a late endosomal/lysosomal member of the CLC family of chloride channels and transporters, or in its beta-subunit Ostm1 cause osteopetrosis and lysosomal storage disease in mice and humans. The severe phenotype of mice globally deleted for ClC-7 or Ostm1 and the absence of storage material in cultured cells hampered investigations… (More)
DOI: 10.1096/fj.09-130880

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