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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]).
-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 