Sulfatase modifying factor 1 trafficking through the cells: from endoplasmic reticulum to the endoplasmic reticulum
| العنوان: | Sulfatase modifying factor 1 trafficking through the cells: from endoplasmic reticulum to the endoplasmic reticulum |
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| المؤلفون: | Maria Chiara Monti, Andrea Ballabio, Maria Pia Cosma, Marianna Cozzolino, Enrico Maria Surace, Thomas Dierks, Mario Buono, Stefano Pepe, Piero Pucci, Ida Annunziata, Ester Zito, Carmine Settembre |
| المساهمون: | Zito, E, Buono, M, Pepe, S, Settembre, Carmine, Annunziata, I, Surace, Enrico Maria, Dierks, T, Monti, Maria, Cozzolino, M, Pucci, Pietro, Ballabio, Andrea, Cosma, Mp |
| المصدر: | The EMBO Journal. 26:2443-2453 |
| بيانات النشر: | Wiley, 2007. |
| سنة النشر: | 2007 |
| مصطلحات موضوعية: | Glycosylation, 0211 other engineering and technologies, 02 engineering and technology, SUMF1, 010501 environmental sciences, Biology, Endoplasmic Reticulum, 01 natural sciences, Article, General Biochemistry, Genetics and Molecular Biology, Cell membrane, Mice, Blood serum, protein secretion and uptake, trafficking, Multiple sulfatase deficiency, Chlorocebus aethiops, medicine, Animals, Humans, Oxidoreductases Acting on Sulfur Group Donors, Secretion, Molecular Biology, Cells, Cultured, 0105 earth and related environmental sciences, 021110 strategic, defence & security studies, General Immunology and Microbiology, Activator (genetics), General Neuroscience, Endoplasmic reticulum, Sulfatase, Cell Membrane, Fibroblasts, medicine.disease, Cell biology, Protein Transport, medicine.anatomical_structure, Biochemistry, COS Cells, Sulfatase-Modifying Factor 1, Sulfatases, Corrigendum, HeLa Cells |
| الوصف: | Sulfatase modifying factor 1 (SUMF1) is the gene mutated in multiple sulfatase deficiency (MSD) that encodes the formylglycine-generating enzyme, an essential activator of all the sulfatases. SUMF1 is a glycosylated enzyme that is resident in the endoplasmic reticulum (ER), although it is also secreted. Here, we demonstrate that upon secretion, SUMF1 can be taken up from the medium by several cell lines. Furthermore, the in vivo engineering of mice liver to produce SUMF1 shows its secretion into the blood serum and its uptake into different tissues. Additionally, we show that non-glycosylated forms of SUMF1 can still be secreted, while only the glycosylated SUMF1 enters cells, via a receptor-mediated mechanism. Surprisingly, following its uptake, SUMF1 shuttles from the plasma membrane to the ER, a route that has to date only been well characterized for some of the toxins. Remarkably, once taken up and relocalized into the ER, SUMF1 is still active, enhancing the sulfatase activities in both cultured cells and mice tissues. |
| وصف الملف: | STAMPA |
| تدمد: | 1460-2075 0261-4189 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::021b82343f6d95a4b0dc6c4f91869a8aTest https://doi.org/10.1038/sj.emboj.7601695Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....021b82343f6d95a4b0dc6c4f91869a8a |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 14602075 02614189 |
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