{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T03:55:51Z","timestamp":1793850951667,"version":"4.6.1"},"reference-count":45,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2009,8,6]],"date-time":"2009-08-06T00:00:00Z","timestamp":1249516800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000780","name":"European Commission","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100001003","name":"Boehringer Ingelheim","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001003","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["faseb.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The FASEB Journal"],"published-print":{"date-parts":[[2009,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Mutations in either ClC\u20107, a late endoso\u2010 mal\/lysosomal member of the CLC family of chloride channels and transporters, or in its \u03b2\u2010subunit Ostm1 cause osteopetrosis and lysosomal storage disease in mice and humans. The severe phenotype of mice globally deleted for ClC\u20107 or Ostm1 and the absence of storage material in cultured cells hampered investiga\u2010 tions of the mechanism leading to lysosomal pathology in the absence of functional ClC\u20107\/Ostm1 transporters. Tissue\u2010specific ClC\u20107\u2010knockout mice now reveal that accumulation of storage material occurs cell\u2010autono\u2010 mously in neurons or renal proximal tubular cells lacking ClC\u20107. Almost all ClC\u20107\u2010deficient neurons die. The activation of glia is restricted to brain regions where ClC\u20107 has been inactivated. The effect of ClC\u20107 disruption on lysosomal function was investigated in renal proximal tubular cells, which display high endo\u2010 cytotic activity. Pulse\u2010chase endocytosis experiments\n                    <jats:italic>in vivo<\/jats:italic>\n                    with mice carrying chimeric deletion of ClC\u20107 in proximal tubules allowed a direct comparison of the handling of endocytosed protein between cells express\u2010 ing or lacking ClC\u20107. Whereas protein was endocytosed similarly in cells of either genotype, its half\u2010life in\u2010 creased significantly in ClC\u20107\u2010deficient cells. These ex\u2010 periments demonstrate that lysosomal pathology is a cell\u2010autonomous consequence of ClC\u20107 disruption and that ClC\u20107 is important for lysosomal protein degrada\u2010 tion.\u2014Wartosch, L., Fuhrmann, J. C., Schweizer, M., Stauber, T., Jentsch, T. J. Lysosomal degradation of endocytosed proteins depends on the chloride trans\u2010 port protein ClC\u20107.\n                    <jats:italic>FASEB J.<\/jats:italic>\n                    23, 4056 \u2013 4068 (2009).\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.fasebj.org\">www.fasebj.org<\/jats:ext-link>\n                  <\/jats:p>","DOI":"10.1096\/fj.09-130880","type":"journal-article","created":{"date-parts":[[2009,8,6]],"date-time":"2009-08-06T21:44:34Z","timestamp":1249595074000},"page":"4056-4068","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":96,"title":["Lysosomal degradation of endocytosed proteins depends on the chloride transport protein ClC\u20107"],"prefix":"10.1096","volume":"23","author":[{"given":"Lena","family":"Wartosch","sequence":"first","affiliation":[{"name":"Leibniz-Institut f\u00fcr Molekulare Pharmakologie (FMP) and Max-Delbr\u00fcck-Centrum f\u00fcr Molekulare Medizin (MDC) Berlin Germany"},{"name":"Freie Universit\u00e4t Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens C.","family":"Fuhrmann","sequence":"additional","affiliation":[{"name":"Leibniz-Institut f\u00fcr Molekulare Pharmakologie (FMP) and Max-Delbr\u00fcck-Centrum f\u00fcr Molekulare Medizin (MDC) Berlin Germany"},{"name":"Metanomics GmbH Tegeler Weg 33 D-10589 Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michaela","family":"Schweizer","sequence":"additional","affiliation":[{"name":"Zentrum f\u00fcr Molekulare Neurobiologie (ZMNH) Universit\u00e4t Hamburg Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tobias","family":"Stauber","sequence":"additional","affiliation":[{"name":"Leibniz-Institut f\u00fcr Molekulare Pharmakologie (FMP) and Max-Delbr\u00fcck-Centrum f\u00fcr Molekulare Medizin (MDC) Berlin 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