The leukocytes of male patients with Fabry's disease are deficient in alpha-galactosidase. The alpha-galactosidase activity in the leukocytes of female carriers of the disease is 15 to 40 percent of the amount present in normal leukocytes. The activities of beta-galactosidase, beta-acetylgalacto...
Fabry's disease (syndrome) [fah´brēz] a sphingolipidosis transmitted as an X-linked recessive trait, in which the glycolipid trihexosyl ceramide is deposited in various tissues, especially the kidneys; the deficient enzyme is α-galactosidase A. It is marked by purpuric skin lesions (angio...
Alpha-galactosidase A; Fabry disease; Hemodialysis; Male Abstract Fabry disease is an X-linked lysosomal storage disorder due to deficient activity of alpha-galactosidase A (α-Gal A) leading to renal insufficiency in males. The aim of present study was to investigate the level of α-Gal A ac...
Alpha-galactosidasedeficiency, orFabry disease[MIM#301500], is the second most common LSD, firstly reported in 1898 [186–188]. Despite being the only X-linked recessivesphingolipidosis, heterozygous women with Fabry disease experience significant life-threatening conditions requiring medical treatment an...
Twitter Google Share on Facebook al·pha (α)-d-ga·lac·to·sid·ase (al'fă gă-lak'tō-sī'dās) An enzyme catalyzing the hydrolysis of α-d-galactosides to release freed-galactose. A deficiency of type A α-d-galactosidase is associated with Fabry disease. ...
Enzyme replacement therapy (ERT) with GLA is currently the most effective therapeutic strategy for patients with Fabry disease, a lysosomal storage disease. 实验方案 Product Specific Protocols IF protocol for CL Plus 488 Alpha Galactosidase A antibody CL488-66121 Download protocol Standard Protocols ...
Alpha-Galactosidase (α-Gal; EC 3.2.1.22) hydrolyzes alpha-galactosyl moieties found in glycolipids and glycoproteins. In mammals, α-Gal hydrolyzes poly- and oligosaccharides commonly found in dietary sources that are difficult to digest. Therefore, α-
Enzyme replacement therapy (ERT) with GLA is currently the most effective therapeutic strategy for patients with Fabry disease, a lysosomal storage disease. 实验方案 Product Specific Protocols WB protocol for Alpha Galactosidase A antibody 66121-1-Ig Download protocol IHC protocol for Alpha Galactosidase...
Galactosidase英 /ɡə'læktosɪ,des; ɡə'læktosɪ,dez; ɡə'læk'tosɪ,des/ 释义n. 牛乳糖;半乳糖苷酶 例句In this paper, the method was based on the hydrolysis of lactose by a β-galactosidase and the liberated glucose was determined.该法基于乳糖酶对乳糖的水解反应,测...
Alpha-galactosidase (Agalsidase) enzyme. Cause of Fabry's disease. Administered as enzyme replacement therapy. Atoms shown as spheres with conventional color coding: hydrogen (white) etc,站酷海洛,一站式正版视觉内容平台,站酷旗下品牌.授权内容包含正版商业