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Differential sorting of lysosomal enzymes in mannose 6-phosphate receptor-deficient fibroblasts.

1994; Springer Nature; Volume: 13; Issue: 15 Linguagem: Inglês

10.1002/j.1460-2075.1994.tb06648.x

1460-2075

Thomas Ludwig, Hélène Munier‐Lehmann, Ulrike Bauer, Michael Hollinshead, Catherine E. Ovitt, Peter Lobel, Bernard Hoflack,

Cellular transport and secretion

Research Article1 August 1994free access Differential sorting of lysosomal enzymes in mannose 6-phosphate receptor-deficient fibroblasts. T. Ludwig T. Ludwig European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author H. Munier-Lehmann H. Munier-Lehmann European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author U. Bauer U. Bauer European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author M. Hollinshead M. Hollinshead European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author C. Ovitt C. Ovitt European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author P. Lobel P. Lobel European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author B. Hoflack B. Hoflack European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author T. Ludwig T. Ludwig European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author H. Munier-Lehmann H. Munier-Lehmann European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author U. Bauer U. Bauer European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author M. Hollinshead M. Hollinshead European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author C. Ovitt C. Ovitt European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author P. Lobel P. Lobel European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author B. Hoflack B. Hoflack European Molecular Biology Laboratory, Heidelberg, Germany. Search for more papers by this author Author Information T. Ludwig1, H. Munier-Lehmann1, U. Bauer1, M. Hollinshead1, C. Ovitt1, P. Lobel1 and B. Hoflack1 1European Molecular Biology Laboratory, Heidelberg, Germany. The EMBO Journal (1994)13:3430-3437https://doi.org/10.1002/j.1460-2075.1994.tb06648.x PDFDownload PDF of article text and main figures. ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinked InMendeleyWechatReddit Figures & Info In higher eukaryotes, the transport of soluble lysosomal enzymes involves the recognition of their mannose 6-phosphate signal by two receptors: the cation-independent mannose 6-phosphate/insulin-like growth factor II receptor (CI-MPR) and the cation-dependent mannose 6-phosphate receptor (CD-MPR). It is not known why these two different proteins are present in most cell types. To investigate their relative function in lysosomal enzyme targeting, we created cell lines that lack either or both MPRs. This was accomplished by mating CD-MPR-deficient mice with Thp mice that carry a CI-MPR deleted allele. Fibroblasts prepared from embryos that lack the two receptors exhibit a massive missorting of multiple lysosomal enzymes and accumulate undigested material in their endocytic compartments. Fibroblasts that lack the CI-MPR, like those lacking the CD-MPR, exhibit a milder phenotype and are only partially impaired in sorting. This demonstrates that both receptors are required for efficient intracellular targeting of lysosomal enzymes. More importantly, comparison of the phosphorylated proteins secreted by the different cell types indicates that the two receptors may interact in vivo with different subgroups of hydrolases. This observation may provide a rational explanation for the existence of two distinct mannose 6-phosphate binding proteins in mammalian cells. Previous ArticleNext Article Volume 13Issue 151 August 1994In this issue RelatedDetailsLoading ...