Human Glucosidase Alpha, Acid (GaA) ELISA Kit | GaA elisa kit

Human Glucosidase Alpha, Acid (GaA) ELISA Kit

Cat. Num. AAA2701445

• Gene Names: GAA; LYAG
• Reactivity: Human
• Synonyms: Glucosidase Alpha; Acid (GaA); Human Glucosidase Alpha; Acid (GaA) ELISA Kit; LYAG; Acid Alpha-Glucosidase; Lysosomal Alpha-Glucosidase; Pompe Disease Glycogen Storage Disease Type II; Acid Maltase; Aglucosidase Alfa; GaA elisa kit

• Reactivity: Human
• Specificity: 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.
• Sequence Length: 952

• Assay Type: Double-antibody Sandwich
• Samples: Serum, Plasma, Tissue Homogenates, Cell Lysates and Other Biological Fluids
• Detection Range: 0.156-10ng/mL
• Sensitivity: < 0.067ng/mL
• Application: Enzyme-linked immunosorbent assay for Antigen Detection.
• 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%
• Preparation and Storage: 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.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for GaA elisa kit

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.

NCBI and Uniprot Product Information

• NCBI GI #: 26251857
• NCBI GeneID: 2548
• UniProt Accession #: P10253; Q09GN4; Q14351; Q16302; Q8IWE7
• Molecular Weight: 105,324 Da
• NCBI Official Full Name: Glucosidase, alpha; acid
• NCBI Official Synonym Full Names: glucosidase alpha, acid
• NCBI Official Symbol: GAA
• NCBI Official Synonym Symbols: LYAG
• NCBI Protein Information: lysosomal alpha-glucosidase
• UniProt Protein Name: Lysosomal alpha-glucosidase
• Protein Family: Lysosomal alpha-glucosidase
• UniProt Gene Name: GAA

NCBI Description

This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2016]

Uniprot Description

Essential for the degradation of glycogen in lysosomes (PubMed:1856189, PubMed:7717400, PubMed:14695532, PubMed:18429042). Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans (PubMed:29061980).