Pentosan Polysulfate: Oral Versus Subcutaneous Injection in Mucopolysaccharidosis Type I Dogs
Fig 6
Clusterin expression in the descending aorta of normal, untreated and PPS-treated MPS I dogs.
(A) Immunohistochemical analysis of clusterin, a marker of inflammation, in the descending aorta of untreated MPS I dogs. High-level expression (brown) of clusterin in aortic plaques and the inner and outer borders of the vessel were observed in the untreated dogs (arrows). PPS-treated animals (17 months with daily oral and 12 months with biweekly subQ) did not show plaque formation and exhibited reduced clusterin staining similar to normal. Magnification 2X. (B) Western blot analysis showing reduced clusterin expression in the descending aorta of PPS-treated animals. Two representative animals are shown for each treatment group. Nor = age-matched normal animal, PPS-O = MPS I dogs treated with daily oral PPS for 17 months. PPS-SQ = MPS dogs treated with biweekly subQ with PPS for 12 months.