Western blot analysis of tight junction protein expressions in various brain structures from MPS III patients. (A) In MPS III A patient, occludin expression was decreased in both putamen and hippocampus, whereas there were no differences in cerebellum compared to control. (D) For claudin-5, slight decrease of protein expression was determined in all three CNS structures evaluated. In MPS III D patient, significant decrease of occludin expression was seen only in primary motor cortex (C) and claudin-5 – in hippocampus and cerebellum (F). Bands for occludin (B) and claudin-5 (E) expressions in various brain structures of MPS III A and MPS III D patients and controls are presented. A columns within panels B and E are bands for control, B columns within panels B and E are bands for MPS III A or III D. GAPDH was used as a normalizing protein.