Figure 5From: Abnormal autophagy, ubiquitination, inflammation and apoptosis are dependent upon lysosomal storage and are useful biomarkers of mucopolysaccharidosis VINormal autophagy, ubiquitination, mitochondrial function, absence of inflammation, and apoptosis in MPS VI CNS without significant DS storage. (A) Quantitative measurement of glycosaminoglycan (GAG) accumulation via dimethyl-methylene blue method on brain lysates of 6-month-old affected (AF) and normal (NR) rats. (GAG levels are expressed in μg per mg of proteins: μg GAG/mg prot). (B) Toluidine blue staining of semi-thin sections (1 μm thick) of brain from 6-month-old AF and NR rats. Magnification: 100×. (C) Quantification of western blots performed on brain lysates from 6-month-old NR and AF rats. Values are expressed as fold of increase compared with NR rat brains (NR = 1) and are the mean ± standard error of three independent experiments. Western blot with anti-p62 antibodies did not allow the detection of any band in the brain of either NR or AF rats. (D) Immuno-histochemical analysis with anti-CD68 antibodies and (E) in situ TUNEL analysis of brain sections from 6-month-old NR and AF rats. Magnification: 100× and 20×, respectively. NS = not statistically significant; OB = olfactory bulb; MB = middle brain; BrSt = brain stem; Hipp = hippocampus; CB = cerebellum.Back to article page