Figure 4.

Alterations in different stages of autophagy in the pathogenesis of lipid storage diseases. Lysosomal lipid storage leads to a reduced ability to autophagosome formation, maturation, or fusion of lysosomes with autophagosomes. This results in a block of the autophagic flux. The steps of these abnormalities are presented in blue boxes. Consequently autophagy substrates (orange boxes) such as protein aggregates and dysfunctional mitochondria accumulate and promote cell death. The inflammatory response, cellular damage or neurodegeneration (orange boxes) further contribute to cell death (red box).