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N. Parkinson, PhD^*, P. G. Ince, MD^*, M. O. Smith, BSc, R. Highley, PhD, G. Skibinski, PhD, P. M. Andersen, MD, K. E. Morrison, PhD, H. S. Pall, MD, O. Hardiman, PhD, J. Collinge, MD, P. J. Shaw, MD^*, EM C. Fisher, PhD^* on behalf of the MRC Proteomics in ALS Study and the FReJA Consortium

From the MRC Prion Unit (N.P., G.S., J.C.) and Department of Neurodegenerative Disease (J.C., E.M.C.F.), Institute of Neurology, University College London, London, UK; Academic Units of Pathology (P.G.I., M.O.S., R.H.) and Neurology (P.J.S.), University of Sheffield, Sheffield, UK; Department of Neurology and Clinical Neuroscience (P.M.A.), Umeå University Hospital, Umeå, Sweden; Division of Neuroscience (K.E.M., H.S.P.), University of Birmingham, Birmingham, UK; and Department of Neurology (O.H.), Beaumont Hospital, Dublin, Eire.

View larger version (101K): [in a new window]   Figure. Photomicrographs of motor system regions from Patient 1. (A) Motor cortex showing intact Betz cells. (B) Low-power view of spinal anterior horn showing numerous motor neuron compact inclusions typical of ALS. These lesions were immunoreactive both to ubiquitin and p62/sequestosome 1 but negative for tau, neurofilament, and -synuclein. (C) Compact inclusion in a spinal cord motor neuron. (D) Motor cortex showing numerous cell profiles immunoreactive for p62/sequestosome1. (E) At high power, these cortical inclusion bodies show coiled body morphology. They were not demonstrated by conventional ubiquitin immunocytochemistry or to antibodies to tau, neurofilament, and -synuclein. Double-labeling immunocytochemistry was performed using either SMI32 (neurons), glial fibrillary acidic protein (astrocytes), CD68 (microglia), or carbonic anhydrase II (oligodendroglia) and p62/sequestosome 1 to identify the cell type involved by coiled body inclusion formation. (F) Coiled bodies immunostained for p62/sequestosome 1. (G) As in F under fluorescence illumination showing cell profiles stained by carbonic anhydrase II. (H) Merged images of parts F and G showing colocalization of coiled bodies to oligodendroglia. (Cresyl fast violet [A], immunoperoxidase [3,3'-diamino-benizidine chromogen] forp62/sequestosome 1 [B, C, D, E, F, H]; immunofluorescence [Texas Red for carbonic anhydrase II] [G, H]; magnification: x2 [B,D]; x40 [A, C, E, F, G, H]).