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Mutant neuroserpin is retained within neurons as intracellular inclusions. These inclusions stain positive with PAS (A) and can be seen within the ER on electron microscopy (B). Electron microscopy of the isolated inclusions confirms that the mutant neuroserpin forms bead-like polymers identical to those of Z α1-antitrypsin (C). Figure reproduced with permission from Lomas et al.97
