Fluorescence polarization analysis, lipid composition, and Na super(+), K super(+)-ATPase kinetics of synaptosomal membranes in feline GM sub(1) and GM sub(2) gangliosidosis

Neurochemical studies were performed on synaptosomal membranes from cats with GM sub(1) or GM sub(2) gangliosidosis to examine possible mechanisms of neuronal dysfunction in these disorders. The basic hypothesis tested was that deficient ganglioside catabolism causes increased ganglioside content of synaptosomal plasma membrane which in turn disrupts normal function. The observations provide evidence for a pathogenetic mechanism of neuronal dysfunction in the gangliosidoses while demonstrating protection of certain vital functional components, such as Na super(+), K super(+)-ATPase.