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Figure 3 | PathoGenetics

Figure 3

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

Figure 3

Impaired autophagy, increased polyubiquitination and dysfunctional mitochondria in visceral organs of MPS VI rats. (A) Electron-microscopic analysis of liver cells from 6-month-old normal (NR) or affected (AF) rats. Arrows, arrowheads and 'x' symbols point at autophagic vescicles (AVs) with different morphology (see Results for description). Asterisks indicate accumulated mitochondria with altered morphology, which are sometimes included in AVs. Scale bars = 2.2 μm. (B) Representative western blot analysis of tissue lysates from NR and AF rat liver, spleen, and kidney blotted with anti -BCN1, -LC3, -ubiquitin, -p62, -COX IV and -actin antibodies. (C) Quantification of western blot analyses. Values are expressed as fold of increase compared with NR rat tissues (NR = 1), and are the mean ± standard error of three independent experiments. (D) Immuno-histochemical analysis with anti-ubiquitin antibodies and (E) immuno-fluorescence analyses with anti-p62 antibodies of liver, spleen, and kidney sections from 6-month-old NR and AF rats. Magnification 100×. *P ≤ 0.05, **P ≤ 0.01, ***P ≤ 0.005.

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