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Mouse anti alpha-Glucosidase
Mouse anti alpha-Glucosidase
- 中文名称：
- Mouse anti alpha-Glucosidase
- 英文名称：
- Mouse anti alpha-Glucosidase
- 品牌：
- AAA Biotech
- 品牌介绍：
- AAA Biotech专注于为全球生命科学研究提供高品质的蛋白质研究工具，核心产品包括经严格验证的抗体、重组蛋白及ELISA试剂盒。
- 货号：
- AAA77501
- 规格：
- 0.1 mg|2x0.1 mg|3x0.1 mg|4x0.1 mg|5x0.1 mg
- 保存建议：
- Store at 4 degree C, or in small aliquots at -20 degree C.
- 货期：
- 6-8周
- 来源宿主：
- Mouse (Balb/c)
- 反应种属：
- Human
- 应用：
- IB (Immunoblot), IHC (Immunohistochemistry)
- 免责声明：
- *本产品仅供科研实验使用，不得用于临床诊断。*
- 其他：
克隆性:Monoclonal
同型:IgG2a
克隆号:[43G7]
特异性:Reacts with the two major bands with apparent molecular weights of 76 000 and 70 000, the band with a molecular weight of about 94000 and minor bands with apparent molecular weights of less than 67 000, when analyzing a-glucosidase isolated from placenta by polyacrylamide gel electrophoresis in the presence of SDS and a reducing agent.
纯度:N/A
形式：Each vial contains 100 ul 1 mg/ml purified monoclonal antibody in PBS containing 0.09% sodium azide.
浓度：N/A
- 说明书：
Lysosomal alpha -glucosidase, like all other lysosomal enzymes of which the 'life-cycle' has been studied, is synthesized as a large precursor that is processed to mature forms of lower molecular mass. Acid alpha -glucosidase Catalyzes the hydrolysis of the alpha1 -> 4 and alpha1 -> 6 glucosidic linkages in glycogen and the alpha1 -> 4 glucosidic linkage in maltose and artificial substRates likep-nitrophenyl- alpha -glucoside. The enzyme is deficient in patients with Glycogenosis Type II (Pompe's disease). Pompe disease (also called Glycogen storage disease type II (GSD II) or acid maltase deficiency) is an autosomal recessive metabolic disorder which damages muscle and nerve cells throughout the body. It is caused by an accumulation of glycogen in the lysosome due to deficiency of the lysosomal acid alpha-glucosidase enzyme. It is the only glycogen storage disease with a defect in lysosomal metabolism. The build-up of glycogen causes progressive muscle weakness (myopathy) throughout the body and affects various body tissues, particularly in the heart, skeletal muscles, liver and nervous system. There are exceptions but levels of alpha-glucosidase determines the type of GSD II an individual may have. More alpha glucosidase present in the individuals muscles means symptoms occur later in life and progress more slowly. GSD II is broadly divided into two onset forms based on the age symptoms occur: Infantile-onset form is usually identified at 4-8 months; muscles appear normal but are limp and weak preventing them from lifting their head or rolling over. As the disease progresses heart muscles thicken and progressively fail. Without treatment death usually occurs due to heart failure and respiRatory weakness.Late/later onset form occurs later than one to two years and progresses more slowly than Infantile-onset form. One of the first symptoms is a progressive decrease in muscle strength starting with the legs and moving to smaller muscles in the trunk and arms, such as the diaphragm and other muscles required for breathing. RespiRatory failure is the most common cause of death. Enlargement of the heart muscles and rhythm disturbances are not significant features but do occur in some casesThe disease is caused by a mutation in a gene (acid alpha-glucosidase: also known as acid maltase) on long arm of chromosome 17 at 17q25.2-q25.3 (base pair 75,689,876 to 75,708,272). The number of mutations described is currently (in 2010) 289 with 67 being non-pathogenic mutations and 197 pathogenic mutations. The remainder are still being evaluated for their association with disease.
