{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T19:19:19Z","timestamp":1789672759899,"version":"4.0.1"},"reference-count":0,"publisher":"Portland Press Ltd.","issue":"2","content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1993,10,15]]},"abstract":"<jats:p>Protein catabolism in fibroblasts cultured from the skin of normal individuals and of patients with mucolipidosis II (I-cell disease) and several other lysosomal storage diseases was examined by metabolic labelling with [3H]leucine and following the fate of radioactive proteins in pulse-chase experiments. In mucolipidosis II cells, overall protein degradative rates were found to be distinctly lower than in normal control cells. To distinguish lysosomal from non-lysosomal degradation, labelling experiments were carried out in the presence and absence of 10 mM NH4Cl, an inhibitor of lysosomal function. It was found that mucolipidosis II fibroblasts exhibited a markedly reduced rate of lysosomal protein degradation, whereas the rate of nonlysosomal degradation appeared normal. Serum and amino acid starvation led to a marked increase in lysosomal protein degradation in normal cells, but had only a minimal effect on that in mucolipidosis II fibroblasts. The specific activities of cathepsins B, H and L were profoundly diminished in all mucolipidosis II cell lines tested. Lysosomal protein degradation in a mucolipidosis III cell line was impaired to a similar degree as in mucolipidosis II cells, whereas it was decreased to a lesser extent in fibroblasts from patients with mucopolysaccharidoses I and VI, galactosialidosis and GM1-gangliosidosis. We conclude that fibroblasts from patients with mucolipidosis II and III have a severely compromised capacity for endogenous lysosomal protein degradation that appears to result from multiple cathepsin deficiency. This lysosomal defect is likely to have pathophysiological consequences.<\/jats:p>","DOI":"10.1042\/bj2950577","type":"journal-article","created":{"date-parts":[[2015,8,10]],"date-time":"2015-08-10T21:35:01Z","timestamp":1439242501000},"page":"577-580","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Protein catabolism in fibroblasts cultured from patients with mucolipidosis II and other lysosomal disorders"],"prefix":"10.1042","volume":"295","author":[{"given":"J","family":"Kopitz","sequence":"first","affiliation":[{"name":"Institute of Pathochemistry and General Neurochemistry, University of Heidelberg, Im Neuenheimer Feld 220\/221, D-69120 Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A","family":"Arnold","sequence":"additional","affiliation":[{"name":"Institute of Pathochemistry and General Neurochemistry, University of Heidelberg, Im Neuenheimer Feld 220\/221, D-69120 Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T","family":"Meissner","sequence":"additional","affiliation":[{"name":"Institute of Pathochemistry and General Neurochemistry, University of Heidelberg, Im Neuenheimer Feld 220\/221, D-69120 Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Cantz","sequence":"additional","affiliation":[{"name":"Institute of Pathochemistry and General Neurochemistry, University of Heidelberg, Im Neuenheimer Feld 220\/221, D-69120 Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","container-title":["Biochemical Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/portlandpress.com\/biochemj\/article-pdf\/295\/2\/577\/611827\/bj2950577.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/portlandpress.com\/biochemj\/article-pdf\/295\/2\/577\/611827\/bj2950577.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,11,23]],"date-time":"2021-11-23T20:59:54Z","timestamp":1637701194000},"score":1,"resource":{"primary":{"URL":"https:\/\/portlandpress.com\/biochemj\/article\/295\/2\/577\/30146\/Protein-catabolism-in-fibroblasts-cultured-from"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1993,10,15]]},"references-count":0,"journal-issue":{"issue":"2","published-print":{"date-parts":[[1993,10,15]]}},"URL":"https:\/\/doi.org\/10.1042\/bj2950577","relation":{},"ISSN":["0264-6021","1470-8728"],"issn-type":[{"value":"0264-6021","type":"print"},{"value":"1470-8728","type":"electronic"}],"subject":[],"published":{"date-parts":[[1993,10,15]]}}}