GLA/alpha-Galactosidase A Proteins, Antibodies, cDNA Clones, ELISA Kits Research Reagents

All GLA/alpha-Galactosidase A reagents are produced in house and quality controlled, including 10 GLA/alpha-Galactosidase A Antibody, 1 GLA/alpha-Galactosidase A ELISA, 26 GLA/alpha-Galactosidase A Gene, 1 GLA/alpha-Galactosidase A IP Kit, 2 GLA/alpha-Galactosidase A Lysate, 2 GLA/alpha-Galactosidase A Protein, 2 GLA/alpha-Galactosidase A qPCR. All GLA/alpha-Galactosidase A reagents are ready to use.

All GLA/alpha-Galactosidase A Reagents

GLA/alpha-Galactosidase A Protein (2)

    GLA/alpha-Galactosidase A Antibody (10)

      GLA/alpha-Galactosidase A ELISA キット(すぐに使用できます)& ELISA抗体ペアセット(すぐに使用できません)(1)

      GLA/alpha-Galactosidase A cDNA Clone (26)

      NM_000169.2

      クローニングベクター cDNA 製品

      In lentiviral vector

      NM_013463.2

      クローニングベクター cDNA 製品

      In lentiviral vector

      GLA/alpha-Galactosidase A Lysate (2)

        GLA/alpha-Galactosidase A の背景知識

        Alpha-galactosidase A, also known as Alpha-D-galactoside galactohydrolase, Alpha-D-galactosidase A, Melibiase and GLA, is a member of the glycosyl hydrolase 27 family. GLA is used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. Defects in GLA are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Deficiency of GLA leads to the accumulation of glycosphingolipids in the vasculature leading to multiorgan pathology. In addition to well-described microvascular disease, deficiency of GLA is also characterized by premature macrovascular events such as stroke and possibly myocardial infarction.

        GLA/alpha-Galactosidase A の参考文献

        • Koide T.et al., 1990, FEBS Lett. 259:353-356.
        • Yang C.-C. et al., 2003, Clin. Genet. 63:205-209.
        • Verovnik F. et al.,2004, Eur. J. Hum. Genet. 12:678-681.
        • Nance C.S. et al., 2006, Arch. Neurol. 63:453-457.

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