Ataxin-3 is transported into the nucleus and associates with the nuclear matrix
Autor: | Danilo Tait, Massimo Riccio, Spartaco Santi, Eberhard Scherzinger, Erich E. Wanker, Nadir M. Maraldi, Andrea Ognibene, Annie Sittler, Hans Lehrach |
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Rok vydání: | 1998 |
Předmět: |
EXPRESSION
PROTEINS Glutamine Recombinant Fusion Proteins Nuclear Localization Signals MACHADO-JOSEPH-DISEASE CELLS LOCALIZATION GENE POLYGLUTAMINE PURIFICATION Nerve Tissue Proteins Importin Biology Cell Fractionation Transfection Antibodies Escherichia coli Tumor Cells Cultured Genetics medicine Animals Humans Nuclear Matrix Nuclear protein Ataxin-3 Molecular Biology Genetics (clinical) Cell Nucleus Microscopy Confocal Nuclear Proteins Biological Transport General Medicine Subcellular localization Nuclear matrix Cell biology Repressor Proteins Cell nucleus medicine.anatomical_structure Microscopy Fluorescence Cytoplasm COS Cells Immunology Nucleus Nuclear localization sequence |
Zdroj: | Human Molecular Genetics. 7:991-997 |
ISSN: | 1460-2083 |
Popis: | It has been reported that the ataxin-3 protein containing a polyglutamine sequence in the pathological range (61-84Q) is localized within the nucleus of neuronal cells, whereas ataxin-3 with a normal repeat length (12-37Q) is predominantly a cytoplasmic protein. In this study, the subcellular localization of the full-length ataxin-3 protein with a glutamine sequence in the normal range (Q3KQ22) was analysed in two mammalian cell lines. Using two affinity-purified polyclonal antibodies raised against the N- or C-terminal portion of ataxin-3, the protein was detected predominantly, but not exclusively, in the nucleus of COS-7 as well as neuroblastoma cells by immunofluorescence and confocal laser scanning microscopy (CLSM). The distribution of the protein in these cellular compartments was confirmed by biochemical subcellular fractionations. Furthermore, CLSM revealed that the ataxin-3 protein present in the nucleus of neuroblastoma cells is associated with the inner nuclear matrix. Our results taken together with the finding of a nuclear localization signal in ataxin-3 indicate that the ataxin-3 protein per se translocates to the nucleus and that an expanded glutamine repeat is not essential for this transport. |
Databáze: | OpenAIRE |
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