Reduction of autophagic, ubiquitination, and mitochondrial anomalies in livers of MPS VI rats following arylsulfatase B gene transfer and DS normalization. (A) Representative western blot analysis of tissue lysates from livers of 6-month-old NR and MPS VI rats treated (TR) or not (AF) with AAV. Membranes were blotted with anti-BCN1, -LC3, -ubiquitin, -p62, -COX IV and -actin antibodies. (B) Quantification of western blots. Values are expressed as fold of increase compared with NR rat tissues (NR = 1) and are the mean of three independent experiments. *P ≤ 0.05, **P ≤ 0.01.