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. Author manuscript; available in PMC: 2014 May 7.
Published in final edited form as: Nat Genet. 2012 Dec 9;45(1):83–87. doi: 10.1038/ng.2497

Figure 3. Autophagy is blocked at a late stage in Vici syndrome.

Figure 3

Accumulation of autophagy adaptors Nbr1, p62/SQSTM1 and the phagophore membrane component LC3- is induced in control and Vici-patient fibroblasts (Patient 4.1) by 12 h treatment with rapamycin, or dual treatment with rapamycin and bafilomycin. In control cells, rapamycin induces slight accumulation of unprocessed LC3-I and processed LC3-II, Nbr1 and p62/SQSTM1; dual induction of autophagy by rapamycin and block of autophagosomal degradation by bafilomycin leads to marked accumulation of all autophagosome components. In Vici patient fibroblasts, strong baseline accumulation of all components is visible that is largely unresponsive to both drugs.