Related Product Information for anti-Trad_1739 antibody
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.
Product Categories/Family for anti-Trad_1739 antibody
References
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.
Product Notes
The Trad_1739 (Catalog #
AAA570206) is an Antibody produced from Mouse (Balb/c) and is intended for research purposes only. The product is available for immediate purchase. The Mouse anti alpha-Glucosidase reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's alpha-Glucosidase can be used in a range of immunoassay formats including, but not limited to, Immunohistochemistry (IHC) (paraffin), Immunoblotting (IB). 43G7 is useful for paraffin-embedded tissues and Western blots. Optimal antibody dilution should be determined by titRation. Researchers should empirically determine the suitability of the Trad_1739 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process.
It is sometimes possible for the material contained within the vial of
"alpha-Glucosidase, Monoclonal Antibody" to become dispersed throughout the inside of
the vial, particularly around the seal of said vial, during shipment and storage. We always
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to consolidate all of the liquid away from the lid and to the bottom of the vial prior to
opening. Please be advised that
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