arylsulfatase A

Arylsulfatase 是原核生物和真核生物的一类酶。Arylsulfatase 是一种硫酸酯酶，常用于生化研究。

Sulfatides are endogenous sulfoglycolipids with various biological activities in the central and peripheral nervous systems, pancreas, and immune system. They are produced from the combination of ceramide and UDP-galactose in the endoplasmic reticulum followed by sulfation in the Golgi apparatus. The ceramide portion contains variable fatty acid chain lengths, which are tissue- and pathology-dependent. Sulfatides are primarily found in the myelin sheath of oligodendrocytes and Schwann cells, with smaller chain lengths predominant during development and longer chain lengths predominant in mature cells. They accumulate in the lysosome of patients with metachromatic leukodystrophy, a disorder characterized by arylsulfatase A deficiency. Sulfatides are also located in pancreatic β-cells and inhibit insulin release from isolated rat pancreatic islet cells, suggesting a potential role in diabetes. Sulfatides can induce inflammation in glia in vitro and certain sulfatides, such as C24:1 3'-sulfo-galactosylceramide, can induce an immune response in vitro in mouse splenocytes. Sulfatides (bovine) (sodium salt) is a mixture of isolated bovine sulfatides.

C17 Ceramide is a synthetic ceramide that contains a non-natural C17:0 fatty acid acylated to sphingosine. It has been used as a marker of arylsulfatase A and sphingolipid activator B activity in patients undergoing enzyme replacement therapy for the genetic disease metachromatic leukodystrophy.

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.

C18 3'-sulfo Galactosylceramide is a member of the sulfatide class of glycolipids. Levels of short-chain sulfatides, including C18 3'-sulfo galactosylceramide, decrease with age in mice and humans. It is increased in brain from mice with an arylsulfatase A deficiency (ASA-KO), particularly in lipid raft fractions. Plasma levels of C18 3'-sulfo galactosylceramide positively correlate with disability progression in patients with relapsing-remitting multiple sclerosis using the Expanded Disability Status Scale. It is also increased in plasma from patients with metachromatic leukodystrophy (MLD).

Tetracosanoyl-sulfatide is an endogenous sulfated glycolipid, which are also known as sulfatides . Tetracosanoyl-sulfatide is the major sulfatide in mature myelin in the central and peripheral nervous systems. Levels of Tetracosanoyl-sulfatide are elevated in plasma derived from patients with metachromatic leukodystrophy, a disorder characterized by arylsulfatase A deficiency, leading to sulfatide accumulation. Unlike C24:1 3'-sulfo galactosylceramide, it does not induce an immune response in mouse splenocytes in vitro.

Arylsulfatase G是一种溶酶体硫酸酯酶，与所有硫酸酯酶，尤其是芳基硫酸酯酶在序列上具有高度相似性。Arylsulfatase G可用于代谢研究。