caa a22 4500 397574541 CHVBK 20180308164851.0 cr unu---uuuuu 161202e199604 xx s 000 0 eng 10.1093/hmg/5.4.481 doi (NATIONALLICENCE)oxford-10.1093/hmg/5.4.481 Partial Characterisation of Murine Huntingtin and Apparent Variations in the Subcellular Localisation of Huntingtin in Human, Mouse and Rat Brain [Elektronische Daten] [Jonathan D. Wood, John C. MacMillan, Peter S. Harper, Pedro R. Lowenstein, A. Lesley Jones] Huntington's disease (HD) is an inherited neurodegenerative disorder caused by the expansion of a CAG repeat in a gene coding for a protein of unknown function. We have raised a polyclonal antibody against a 12 amino acid peptide (residues 2110-2121 of human huntingtin) which specifically recognises huntingtin on Western blots of human, rat and mouse brain. We have characterised huntingtin expression in the mouse. The protein was detected on Western blots of all mouse tissues examined, with the highest expression seen in brain. Human, mouse and rat brain were fractionated by differential centrifugation and discontinuous Percoll gradients. The fractions were analysed by Western blotting for huntingtin and synaptophysin (a synaptic vesicle localised protein). In mouse brain, huntingtin was localised in the soluble S3 fraction; in rat brain it was localised in the soluble S3 fraction and also in the membrane P2 and P3 fractions; in both normal and HD-affected human brain, huntingtin was membrane bound with a distribution essentially the same as that of synaptophysin. These observed differences in the subcellular localisation of huntingtin between mouse and human brain are important in the context of mouse models for HD. © 1996 Oxford University Press Wood Jonathan D. Institute of Medical Genetics, University of Wales College of Medicine, Heath Park, Cardiff CF4 4XN, UK aut MacMillan John C. Institute of Medical Genetics, University of Wales College of Medicine, Heath Park, Cardiff CF4 4XN, UK aut Harper Peter S. Institute of Medical Genetics, University of Wales College of Medicine, Heath Park, Cardiff CF4 4XN, UK aut Lowenstein Pedro R. Department of Physiology, School of Molecular and Medical Biosciences, University of Wales College of Cardiff, Cardiff, UK aut Jones A. Lesley Institute of Medical Genetics, University of Wales College of Medicine, Heath Park, Cardiff CF4 4XN, UK aut Human Molecular Genetics Oxford University Press 5/4(1996-04), 481-487 0964-6906 5:4<481 1996 5 hmg https://doi.org/10.1093/hmg/5.4.481 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1093/hmg/5.4.481 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Wood Jonathan D. Institute of Medical Genetics, University of Wales College of Medicine, Heath Park, Cardiff CF4 4XN, UK aut NATIONALLICENCE 700 1- MacMillan John C. Institute of Medical Genetics, University of Wales College of Medicine, Heath Park, Cardiff CF4 4XN, UK aut NATIONALLICENCE 700 1- Harper Peter S. Institute of Medical Genetics, University of Wales College of Medicine, Heath Park, Cardiff CF4 4XN, UK aut NATIONALLICENCE 700 1- Lowenstein Pedro R. Department of Physiology, School of Molecular and Medical Biosciences, University of Wales College of Cardiff, Cardiff, UK aut NATIONALLICENCE 700 1- Jones A. Lesley Institute of Medical Genetics, University of Wales College of Medicine, Heath Park, Cardiff CF4 4XN, UK aut NATIONALLICENCE 773 0- Human Molecular Genetics Oxford University Press 5/4(1996-04), 481-487 0964-6906 5:4<481 1996 5 hmg Metadata rights reserved CC BY-NC-4.0 http://creativecommons.org/licenses/by-nc/4.0 nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-oxford