BACE1-cleavage of Sez6 and Sez6L is elevated in Niemann-Pick type C disease mouse brains
Fig 3
At 10-weeks of age an enhanced BACE1-mediated proteolysis of Sez6L is present in all three brain regions of NPC1 vs. wt mice, while cleaved Sez6 is increased only in the cerebellum.
(A-C) Western blot analyses of soluble Sez6 (sSez6), soluble Sez6L (sSez6L), total soluble APP (sAPPt) and actin (Actin-DEA) in DEA fractions of the cortex (A), hippocampus (B) and cerebellum (C) collected from 10-weeks old wt (NPC1+/+; N = 6) and NPC1 (NPC1-/-; N = 6) mice. A standard protein lysate (S) was included twice on each gel. (D-F) Graphs representing quantified protein signals of sSez6 (D), sSez6L (E) and sAPPt (F) which were normalized against actin (Actin-DEA) in the cortex (CX), hippocampus (HP) and cerebellum (CB) of 10-weeks old wt (NPC1+/+; N = 6) and NPC1 (NPC1-/-; N = 6) mice.