Ataxin-7 inclusions in enteric neurons of PrP-SCA7-92Q mice. (A) Human ataxin-7-specific antibodies demonstrate punctate brown inclusions in the nuclei of a subset of enteric ganglion cells from a PrP-SCA7-92Q mouse. (B) An immediately adjacent paraffin section immunostained with SUMO-1-specific antisera shows SUMO-1 immunoreactivity in the same nuclei (numbered 1 to 4). (C,D) Wholemount preparations or myenteric plexus immunostained with antibodies that recognize ataxin-7 (red) and the neural marker, Hu (green), demonstrate no intranuclear inclusions in a non-transgenic sample (C), but abundant intranuclear inclusions in a subset of neurons from a transgenic mouse (D). (E) In a paraffin section, ataxin-7 (red) immunoreactivity is present in the nuclei of a subset of myenteric neurons that co-express the cytoplasmic marker, choline acetyltransferase (ChAT, green). (F) In contrast, a wholemount preparation of a myenteric ganglion does not show cellular colocalization of ataxin-7 (red) with neuronal nitric oxide synthase (nNOS, green).