Fig. 2

Immunofluorescence microscopy analysis using antibodies raised against catalase, a peroxisomal matrix enzyme, in skin fibroblasts of a control subject (left panels) and the patient, F1453 (right panels) cultured at 37 °C (upper panels) and 40 °C (lower panels) for 2 weeks. At 37 °C cells of the patient reveal a normal peroxisomal staining in most cells (a representative picture is shown), whereas at 40 °C catalase staining becomes cytosolic confirming the peroxisome biogenesis defect in the patient