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Merck
모든 사진(1)

주요 문서

MAK390

Sigma-Aldrich

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

sufficient for 100 fluorometric tests

동의어(들):

Alpha-Gal Assay Kit

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About This Item

UNSPSC 코드:
12161503

검출 방법

fluorometric

저장 온도

−20°C

일반 설명

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.

애플리케이션


  • 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).

특징 및 장점

The Alpha Galactosidase (α-Gal) Activity Assay Kit:
  • Detection limit: < 0.1μU.
  • Specific: does not detect beta galactosidase activity.

적합성

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.).

원리

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 (λex = 360 nm/λem = 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.

Storage Class Code

10 - Combustible liquids

WGK

WGK 3


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