[HTML][HTML] Metabolic processing of gangliosides by normal and salla human fibroblasts in culture: A study performed by administering radioactive GM3 ganglioside

V Chigorno, G Tettamanti, S Sonnino - Journal of Biological Chemistry, 1996 - ASBMB
Cultured fibroblasts from normal subjects and from subjects affected by Salla disease,
characterized by the lack or misfunction of the membrane carrier responsible for the egress
of sialic acid from lysosomes, were fed with ganglioside G M3 labeled at the sialic acid
acetyl group,[Neu5Ac-3 H] G M3, or at C-3 of sphingosine (Sph),[Sph-3 H] G M3, or at C-1 of
stearoyl chain,[stearoyl-14 C] G M3. After a 15-h pulse the total amount of cell-bound G M3
corresponded to about 2% of the endogenous ganglioside content. Cells were then …