MAK390

Alpha Galactosidase (α-Gal) Activity Assay Kit (Fluorometric)

sufficient for 100 fluorometric tests

• 別名: Alpha-Gal Assay Kit
• UNSPSCコード: 12161503

特性

• 検出方法: fluorometric
• 保管温度: −20°C

詳細

詳細

Alpha-Galactosidase (α-Gal) hydrolyzes alpha-galactosyl moieties found in glycolipids and glycoproteins. In mammals, a-Gal hydrolyzes poly- and oligosaccharides commonly found in dietary sources that are difficult to digest. Therefore, α-Gal is used in dietary supplements that help to reduce the production of intestinal gases due to consumption of certain foods. It is known total α-Gal activity is due to two major isozymes with unique, yet different thermostability profiles. Alpha-Galactosidase A, is thermolabile and represents approximately 90% of total α-Gal activity. Fabry Disease, a lysosomal disease disorder, is characterized by mutations in alpha-Galactosidase A. These mutations cause abnormal accumulation of glycosphingolipids in lysosomes.

アプリケーション

• Exploration of mechanism of total triterpenoids from fruits of Chaenomeles speciosa against senescent GES-1 cells induced by D-galactose based on Gln/GLS1/a-KG metabolic axis and mitochondrial apoptosis signaling pathway.: This study investigates the effects of total triterpenoids on senescent GES-1 cells, exploring mechanisms involving the Gln/GLS1/a-KG metabolic axis and mitochondrial apoptosis signaling pathway (He et al., 2024).


• Diagnosis of Fabry Disease Using Alpha-Galactosidase A Activity or LysoGb3 in Blood Fails to Identify Up to Two Thirds of Female Patients.: This research highlights the limitations of using alpha-galactosidase A activity and LysoGb3 in blood for diagnosing Fabry Disease, especially in female patients (Duro et al., 2024).


• Pegunigalsidase alfa: a novel, pegylated recombinant alpha-galactosidase enzyme for the treatment of Fabry disease.: This review focuses on Pegunigalsidase alfa, a new pegylated recombinant enzyme for treating Fabry Disease, discussing its efficacy and potential benefits (Germain & Linhart, 2024).

特徴および利点

The Alpha Galactosidase (α-Gal) Activity Assay Kit:

• Detection limit: < 0.1μU.
• Specific: does not detect beta galactosidase activity.

適合性

The kit is suitable for the detection of α-Galactosidase activity in tissue homogenates (kidney, etc.), cell lysates (U937, etc.) and biological fluids (saliva, etc.).

原理

The Alpha Galactosidase (α-Gal) Activity Assay Kit provides a simple, rapid way to monitor total α-Gal activity in a wide variety of biological samples. In this kit, α-Gal cleaves a synthetic specific substrate releasing a fluorophore, which can be easily quantified (λ_ex = 360 nm/λ_em = 445 nm). The assay is specific (beta galactosidase activity is not detected), sensitive and can detect as low as 0.1 μU of α-Galactosidase activity.

安全性情報

• 保管分類コード: 10 - Combustible liquids
• WGK: WGK 3