Immunohistochemical detection of GM2 ganglioside in dKO, KIKO and CON brains. Sections were selected from similar areas of the brain for matched pairs. Sections for each matched pair were stained together and photographed at 63× using a Zeiss Axioimager equipped with ZEN software. Many images are taken from within the same field of view as the 20× images shown in Fig. S6A. Specifically, data in Fig. 6, panels E,F,G,I,J,N, and O are also shown in Fig. S6A, panels A,B,C,I,J,N, and O respectively. The presence of a red-brown staining signal for GM2 ganglioside was present in CON brains but much more broadly and abundantly present in the sections from the dKO and KIKO brains. Arrows indicate cells that have low levels of GM2 in CON (A,C,E,F,I,K,M,O), compared to the higher levels of GM2 present in vacuolated cells (B,D,F,H,J,L,N,P). Arrows in panels A-D identify Purkinje cells. Scale bars: 20 µM. These images are representative of those from three different pairs of animals.
Emily Barker & co generate and characterise mouse models of complete and partial HexA deficiency that are suitable for testing a broad range of therapies for Tay-Sachs disease. 🐭 doi.org/10.1242/bio....
#TaySachsDisease #MouseModel