GLB1 and GM1 gangliosidosis: GM1 gangliosidosis occurs due to the GLB1 (in human)/Glb1 (in mouse) gene defects, which is critical for encoding an enzyme β gal (E.C. 3.2.1.23) responsible for the hydrolysis of the GM1, oligosaccharides, and keratan sulfate, all of which are critical for performing many of the cellular signaling and the proper functions of the brain and visceral organs [117,118].