2003
DOI: 10.1016/j.cellbi.2003.07.001
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Deficiency of mannose 6‐phosphate receptors and lysosomal storage: a morphometric analysis of hepatocytes of neonatal mice

Abstract: Transport of lysosomal enzymes is mediated by two mannose 6-phosphate receptors: a cation dependent (CD-MPR) and a cation independent receptor (CI-MPR). In the present study the effect of MPR-deficiency on the lysosomal system of neonatal mouse hepatocytes was studied by ultrastructural morphometric analyses. The volume density of the lysosomal system in hepatocytes of mice that lack both receptors was significantly increased in comparison with controls and with mice deficient for CI-MPR only. This higher volu… Show more

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Cited by 7 publications
(6 citation statements)
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“…However, despite over 20 years of investigation (6,7), the specific role of each MPR in lysosomal protein targeting remains unclear. Studies using mutant mice indicate that animals lacking either the CDMPR (12,13) or the CIMPR (9) exhibit no apparent health defects, whereas the combined MPR deficiency results in a profound lysosomal storage phenotype and early death (10,14). This indicates that each MPR can partially compensate for the absence of the other, which complicates determination of MPR-specific functions.…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…However, despite over 20 years of investigation (6,7), the specific role of each MPR in lysosomal protein targeting remains unclear. Studies using mutant mice indicate that animals lacking either the CDMPR (12,13) or the CIMPR (9) exhibit no apparent health defects, whereas the combined MPR deficiency results in a profound lysosomal storage phenotype and early death (10,14). This indicates that each MPR can partially compensate for the absence of the other, which complicates determination of MPR-specific functions.…”
Section: Discussionmentioning
confidence: 99%
“…CDMPR mutants are fertile and have no obvious defects (12,13). Mice lacking both the CDMPR and CIMPR in an IGF2-deficient background have a short lifespan and exhibit a lysosomal storage phenotype (10,14).…”
mentioning
confidence: 99%
“…Their suggestion that lysosomal enzymes contained a recognition marker required for uptake and transport to lysosomes was later confirmed with the identification of the marker as Man-6-P (Kaplan et al 1977;Distler et al 1979;Natowicz et al 1979). Analyses of fibroblasts derived from transgenic animals lacking both MPRs show that these cells secrete the majority of their lysosomal enzymes and accumulate undigested material in their lysosomes in a manner similar to that observed in fibroblasts from ML II patients (Ludwig et al 1996;Dittmer et al 1998;Schellens et al 2003), demonstrating that a similar phenotype can result from either a deficiency of the Man-6-P tag or the MPRs.…”
Section: Generation Of Man-6-p On N-linked Oligosaccharidesmentioning
confidence: 71%
“…However, impaired GlcNAc-phosphotransferase activity results in the mis-sorting and secretion of numerous lysosomal enzymes due to their inability to acquire Man-6-P residues and be trafficked by the MPRs, causing the LSDs, mucolipidosis II and III (also referred to as “I-cell disease” and “pseudo-Hurler polydystrophy”, respectively) (for reviews see [62, 63]). As anticipated from these findings, fibroblasts derived from transgenic mice lacking both MPRs demonstrate a similar phenotype to ML-II fibroblasts, as evidenced by the secretion of most acid hydrolases and accumulation of undigested matter in the lysosomes of these cells [64-66]. …”
Section: Comparison Of the Carbohydrate Binding Properties Of The mentioning
confidence: 93%