Human Glycogen phosphorylase, muscle form (PYGM) ELISA Kit

Mutations in the muscle isoform of glycogen phosphorylase (PYGM) are associated with McArdle disease (glycogen storage disease type V) . More than 65 mutations in the PYGM gene that lead to McArdle disease have been identified to date.Gautron et al. (1987) isolated muscle phosphorylase cDNA clones from a human cDNA library. Northern blot experiments revealed 1 specific mRNA of 3.4 kb found uniquely in tissues expressing muscle phosphorylase. The muscle glycogen phosphorylase protein comprises 842 amino acids (Kubisch et al., 1998) .Burke et al. (1987) determined the intron/exon structure of the PYGM gene. Kubisch et al. (1998) provided a revised genomic structure for the PYGM gene, which contains 20 exons.

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Human Glycogen phosphorylase, muscle form (PYGM) ELISA Kit has high sensitivity and excellent specificity for detection of Human PYGM. No significant cross-reactivity or interference between Human PYGM and analogues was observed.

This Human Glycogen phosphorylase, muscle form (PYGM) ELISA Kit employs a two-site sandwich ELISA to quantitate PYGM in samples. An antibody specific for PYGM has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyPYGM present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for PYGM is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of PYGM bound in the initial step. The color development is stopped and the intensity of the color is measured.

• Do not mix components from different kit lots or use reagents beyond the kit expiration date.• Allow all reagents to warm to room temperature for at least 30 minutes before opening.• Pre-rinse the pipet tip with reagent, use fresh pipet tips for each sample, standard and reagent to avoid contamination.• Unused wells must be kept desiccated at 4 °C in the sealed bag provided.• Mix Thoroughly is very important for the result. It is recommended using low frequency oscillator or slight hand shaking every 10 minutes.• It is recommended that all samples and standards be assayed in duplicate or triplicate.