Catalogue

Background
Lysosomal α -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 α -glucosidase Catalyzes the hydrolysis of the α1 -> 4 and α1 -> 6 glucosidic linkages in glycogen and the α1 -> 4 glucosidic linkage in maltose and artificial substRates likep-nitrophenyl- α -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 diagnosed 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.

Source
43G7 is a Mouse monoclonal IgG2a* antibody derived by fusion of Mouse myeloma cells with spleen cells from a Balb/cHeA Mouse hyperimmunized with purified acid alpha-glucosidase from Human placenta. * Please note that, based on recent analysis, the isotype of this antibody has been updated

Product
Each vial contains 100 ul 1 mg/ml ProtA/G purified monoclonal antibody in PBS containing 0.09% sodium azide.

Purification Method: The antibody is purified by ProtA/G affinity chromatography.

Specificity
Reacts with the two major bands with apparent molecular weights of 76.000 and 70.000, the band with a molecular weight of about 94.000 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.

Applications
43G7 is useful for paraffin-embedded tissues and Western blots. Optimal antibody dilution should be determined by titration.

Storage
The antibody is shipped at ambient temperature and may be stored at +4°C. For prolonged storage prepare appropriate aliquots and store at or below -20°C. Prior to use, an aliquot is thawed slowly in the dark at ambient temperature, spun down again and used to prepare working dilutions by adding sterile phosphate buffered saline (PBS, pH 7.2). Repeated thawing and freezing should be avoided. Working dilutions should be stored at +4°C, not refrozen, and preferably used the same day. If a slight precipitation occurs upon storage, this should be removed by centrifugation. It will not affect the performance or the concentration of the product.

Shipping Conditions: Ship at ambient temperature.

Caution
This product is intended FOR RESEARCH USE ONLY, and FOR TESTS IN VITRO, not for use in diagnostic or therapeutic procedures involving humans or animals. It may contain hazardous ingredients. Please refer to the Safety Data Sheets (SDS) for additional information and proper handling procedures. Dispose product remainders according to local regulations.This datasheet is as accurate as reasonably achievable, but our company accepts no liability for any inaccuracies or omissions in this information.

References
1. John Hilkens, Joseph M. Tager, Femke Buijs, Betty Brouwer-Kelder, Gerda M. Van Thienen, Frans P.W. Tegelaers and Jo Hilgers (1981): Monoclonal antibodies anti Human acid t-glucosidase, Biochimica et Biophysica Acta, 678 ( 1981) 7-11.

Safety Datasheet(s) for this product: