ELISA/assay

Glucosidase Alpha, Acid (GaA) ELISA Kit

MBS2020915

48-Strip-Wells

515 USD

ELISA Kit

Glucosidase Alpha, Acid (GaA) ELISA Kit

Glucosidase Alpha, Acid (GaA)

LYAG; Acid Alpha-Glucosidase; Lysosomal Alpha-Glucosidase; Pompe Disease Glycogen Storage Disease Type II; Acid Maltase; Aglucosidase Alfa

Glucosidase, alpha; acid; Lysosomal alpha-glucosidase; lysosomal alpha-glucosidase; glucosidase, alpha; acid; Acid maltase; Aglucosidase alfa

GaA

GAA; GAA; LYAG

LYAG_HUMAN

Human

952

This assay has high sensitivity and excellent specificity for detection of Glucosidase Alpha, Acid (GaA). No significant cross-reactivity or interference between Glucosidase Alpha, Acid (GaA) and analogues was observed.

For unopened kit: All the reagents should be kept according to the labels on vials. The Standard, Detection Reagent A, Detection Reagent B and the 96-well strip plate should be stored at -20 degree C upon receipt while the others should be at 4 degree C. For opened kit: When the kit is opened, the remaining reagents still need to be stored according to the above storage condition. Besides, please return the unused wells to the foil pouch containing the desiccant pack, and reseal along entire edge of zip-seal. The stability of kit is determined by the loss rate of activity. The loss rate of this kit is less than 5% within the expiration date under appropriate storage condition. To minimize extra influence on the performance, operation procedures and lab conditions, especially room temperature, air humidity, incubator temperature should be strictly controlled. It is also strongly suggested that the whole assay is performed by the same operator from the beginning to the end.

Samples: Tissue homogenates, Cell lysates and other biological fluids. Assay Type: Sandwich. Detection Range: 0.156-10ng/mL. Sensitivity: Typically less than 0.054ng/mL.

Precision: Intra-assay Precision (Precision within an assay): 3 samples with low, middle and high level Glucosidase Alpha, Acid (GaA) were tested 20 times on one plate, respectively. Inter-assay Precision (Precision between assays): 3 samples with low, middle and high level Glucosidase Alpha, Acid (GaA) were tested on 3 different plates, 8 replicates in each plate. CV(%) = SD/meanX100 . Intra-Assay: CV<10% . Inter-Assay: CV<12% . Assay Procedure Summary: 1. Prepare all reagents, samples and standards;. 2. Add 100uL standard or sample to each well. Incubate 2 hours at 37 degree C;. 3. Aspirate and add 100uL prepared Detection Reagent A. Incubate 1 hour at 37 degree C;. 4. Aspirate and wash 3 times;. 5. Add 100uL prepared Detection Reagent B. Incubate 30 minutes at 37 degree C;. 6. Aspirate and wash 5 times;. 7. Add 90uL Substrate Solution. Incubate 15-25 minutes at 37 degree C;. 8. Add 50uL Stop Solution. Read at 450nm immediately.

Principle of the Assay: The test principle applied in this kit is Sandwich enzyme immunoassay. The microtiter plate provided in this kit has been pre-coated with an antibody specific to Glucosidase Alpha, Acid (GaA). Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated antibody specific to Glucosidase Alpha, Acid (GaA). Next, Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. After TMB substrate solution is added, only those wells that contain Glucosidase Alpha, Acid (GaA), biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450nm 10nm. The concentration of Glucosidase Alpha, Acid (GaA) in the samples is then determined by comparing the O.D. of the samples to the standard curve.

26251857

AAH40431.1

P10253

105,324 Da

Galactose Metabolism Pathway (82931); Galactose Metabolism Pathway (292); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolic Pathways (132956); Notch-mediated HES/HEY Network Pathway (169347); Starch And Sucrose Metabolism Pathway (82974); Starch And Sucrose Metabolism Pathway (344)

This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008]

GAA: Essential for the degradation of glygogen to glucose in lysosomes. Defects in GAA are the cause of glycogen storage disease type 2 (GSD2); also called acid alpha-glucosidase (GAA) deficiency or acid maltase deficiency (AMD). GSD2 is a metabolic disorder with a broad clinical spectrum. The severe infantile form, or Pompe disease, presents at birth with massive accumulation of glycogen in muscle, heart and liver. Cardiomyopathy and muscular hypotonia are the cardinal features of this form whose life expectancy is less than two years. The juvenile and adult forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle failure. Patients with the adult form can be free of clinical symptoms for most of their life but finally develop a slowly progressive myopathy. Belongs to the glycosyl hydrolase 31 family. Protein type: Hydrolase; Carbohydrate Metabolism - galactose; Contractile; EC 3.2.1.20; Carbohydrate Metabolism - starch and sucrose. Chromosomal Location of Human Ortholog: 17q25.2-q25.3. Cellular Component: membrane; lysosomal membrane; lysosome. Molecular Function: alpha-glucosidase activity; maltase activity; carbohydrate binding. Biological Process: heart morphogenesis; tissue development; maltose metabolic process; glycogen catabolic process; vacuolar sequestering; glucose metabolic process; locomotory behavior; sucrose metabolic process; muscle maintenance; neuromuscular process controlling posture; lysosome organization and biogenesis; diaphragm contraction; neuromuscular process controlling balance; regulation of the force of heart contraction; cardiac muscle contraction. Disease: Glycogen Storage Disease Ii

48-Strip-Wells

515 USD

96-Strip-Wells

695

5x96-Strip-Wells

2685

10x96-Strip-Wells

4850