fig3

Figure 3. Dysregulation of mammalian target of rapamycin (mTOR) C1 signaling in LAM pathogenesis. Constitutive activation of the Raptor-containing mTOR leads to dysregulated mTOR signaling pathways in LAM cells. Consequently, cell proliferation, cell growth, lung remodeling as well apoptosis and cell survival are altered. This scheme corresponds to a non-exhaustive summary, emphasizing some key proteins which are specifically linked to mTOR-dependent under-expression or over-expression in LAM cells. Representative upregulated molecules: HIF-1α (hypoxia-inducible factor 1α), VEGF (vascular endothelial growth factor)-C and VEGF-D, MMP (matrix metalloproteinase), IMPDH (inosine 5'-monophosphate dehydrogenase), p70S6K (70 kDa ribosomal protein S6 kinase), SREBP (sterol regulatory element-binding protein) and downregulated molecules: BCL2 (B-cell lymphoma 2), pTFEB (phosphorylated form of transcription factor EB), ULK1 (Unc-51-like autophagy-activating kinase 1).