Skip to main content
Figure 5 | PathoGenetics

Figure 5

From: Abnormal autophagy, ubiquitination, inflammation and apoptosis are dependent upon lysosomal storage and are useful biomarkers of mucopolysaccharidosis VI

Figure 5

Normal 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