Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies
| العنوان: | Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies |
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| المؤلفون: | Donna L. Smith, Gillian P. Bates, Sabine Schipper-Krom, Huib Ovaa, Rianne van den Nieuwendijk, Mark A. Hink, Anne H. P. Jansen, Hermen S. Overkleeft, Eric Reits, Anne Wiemhoefer, Katrin Juenemann |
| المساهمون: | Faculteit der Geneeskunde, Molecular Cytology (SILS, FNWI), Amsterdam institute for Infection and Immunity, Amsterdam Gastroenterology Endocrinology Metabolism, Amsterdam Neuroscience, Cancer Center Amsterdam, Cell Biology and Histology, Other departments, Graduate School, Medical Biochemistry |
| المصدر: | FEBS Letters, 588(1), 151-159. Wiley-Blackwell FEBS letters, 588(1), 151-159. Wiley-Blackwell |
| سنة النشر: | 2013 |
| مصطلحات موضوعية: | Male, Biochemistry, Inclusion bodies, Mice, Ubiquitin, Structural Biology, Inclusion Bodies, Huntingtin Protein, Microscopy, Confocal, biology, Chemistry, Brain, Cell biology, Huntington Disease, Female, Intracellular, Protein Binding, Proteasome Endopeptidase Complex, Huntington, Blotting, Western, Green Fluorescent Proteins, Biophysics, Protein Misfolding Disorder, Mice, Transgenic, Nerve Tissue Proteins, Huntington's disease, Cell Line, Tumor, Genetics, medicine, Animals, Humans, Molecular Biology, Aggregate, Proteasome, Fluorescence recovery after photobleaching, Cell Biology, medicine.disease, Mice, Inbred C57BL, Disease Models, Animal, HEK293 Cells, Mutation, biology.protein, Mice, Inbred CBA, Peptides, Trinucleotide Repeat Expansion, Polyglutamine, HeLa Cells |
| الوصف: | Neurodegenerative disorders such as Huntington’s disease are hallmarked by neuronal intracellular inclusion body formation. Whether proteasomes are irreversibly recruited into inclusion bodies in these protein misfolding disorders is a controversial subject. In addition, it has been proposed that the proteasomes may become clogged by the aggregated protein fragments, leading to impairment of the ubiquitin–proteasome system. Here, we show by fluorescence pulse-chase experiments in living cells that proteasomes are dynamically and reversibly recruited into inclusion bodies. As these recruited proteasomes remain catalytically active and accessible to substrates, our results challenge the concept of proteasome sequestration and impairment in Huntington’s disease, and support the reported absence of proteasome impairment in mouse models of Huntington’s disease. |
| تدمد: | 1873-3468 0014-5793 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5da4810d651cab0f491dd9dc1ddf60acTest https://pubmed.ncbi.nlm.nih.gov/24291262Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....5da4810d651cab0f491dd9dc1ddf60ac |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 18733468 00145793 |
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