IDUA protein immunostaining and mitophagy in head and neck paraganglioma. (a) Immunofluorescence detects cytoplasmic IDUA protein labeling (green) in the neuroepithelial zellballen of paraganglioma. The zellballen are outlined in red by mainly peripheral labeling with antibody to HCAM/CD44, a surface stem cell marker that functions as a receptor for hyaluronan, a glycosaminoglycan degraded by the IDUA product (double immunofluorescence on semithin frozen section, bar = 10 µm). (b) Immunofluorescence highlights spots of colocalization (yellow) between anti-p62 antibody (Sequestosome-1, SQSTM, green) and anti-mitochondrial antibody 113-1 (ABCAM, red) (double immunofluorescence on semithin frozen section, bar = 10 µm). (c) Ultrastructural cross section of an autophagosome (indicated by arrows) containing a swollen mitochondrion, detected in the cytoplasm of a neuroepithelial paraganglioma cell (transmission electron micrograph, bar = 1 µm).