alpha-Glucosidase, ELISA Kit

Goat a-Glucosidase ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 17; NC_000017.10 (78075355..78093679). Location: 17q25.2-q25.3

Store all reagents at 2-8 degree C

We suggest pre-experimenting with neat (undiluted) samples, 1:2 or 1:4 dilutions. Please avoid diluting your samples more than 1:10 as it would exceed the dilution limit set for this kit. If the expected concentration of the target is beyond the detection range of the kit, please contact our technical support team

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of A-Glu elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS745184 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the alpha-Glucosidase, ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing A-Glu. The ELISA analytical biochemical technique of the MBS745184 kit is based on A-Glu antibody-A-Glu antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect A-Glu antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, A-Glu. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Metabolism

105,324 Da

glucosidase, alpha; acid

lysosomal alpha-glucosidase; acid maltase; aglucosidase alfa

Lysosomal alpha-glucosidase

LYAG_HUMAN

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 ***** forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle failure. Patients with the ***** 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: EC 3.2.1.20; Carbohydrate Metabolism - starch and sucrose; Contractile; Carbohydrate Metabolism - galactose; Hydrolase

Chromosomal Location of Human Ortholog: 17q25.2-q25.3

Cellular Component: membrane; lysosome; lysosomal membrane

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

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