3'-sulfo Galactosylsphingosine (ammonium salt)

3'-sulfo Galactosylsphingosine is a form of sulfatide that is lacking the fatty acyl group. It decreases migration and adhesion of B35 neuroblastoma cells and increases cell rounding when used at a concentration of 20 µM. It also inhibits PKC and cytochrome c oxidase activity when used at concentrations of 150 and 50-100 µM, respectively. 3'-sulfo Galactosylsphingosine accumulates in patients with metachromatic leukodystrophy, a lysosomal storage disorder characterized by arylsulfatase A (ASA) deficiency leading to progressive demyelination and neuromotor deficits. In mice lacking ASA, levels of 3'-sulfo galactosylsphingosine increase after one month of age followed by demyelination and neuromotor deficits. 3'-sulfo Galactosylsphingosine has been used as a standard for the quantification of 3'-sulfo galactosylsphingosine by LC-MS.