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Anti-PYGM Antibody Picoband® APC Conjugated

Product Specifications

Background

This gene encodes a muscle enzyme involved in glycogenolysis. Highly similar enzymes encoded by different genes are found in liver and brain. Mutations in this gene are associated with McArdle disease (myophosphorylase deficiency), a glycogen storage disease of muscle. Alternative splicing results in multiple transcript variants.

Synonyms

PYGM; Glycogen phosphorylase, muscle form; EC 2.4.1.1; Myophosphorylase

Gene Name

PYGM

Gene ID

5837

UniProt

P11217

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins

Immunogen

E.coli-derived human PYGM recombinant protein (Position: V390-R804) . Human PYGMshares 95.9% and 94.2% amino acid (aa) sequence identity with mouse and rat PYGM, respectively.

Clonality

Polyclonal

Applications

Flow Cytometry

Purification

Immunogen affinity purified.

Form

Liquid

References & Citations

1. Andreu, A. L., Nogales-Gadea, G., Cassandrini, D., Arenas, J., Bruno, C. McArdle disease: molecular genetic update. Acta Myol. 26: 53-57, 2007. 2. Bartram, C., Edwards, R. H. T., Clague, J., Beynon, R. J. McArdle's disease: a nonsense mutation in exon 1 of the muscle glycogen phosphorylase gene explains some but not all cases. Hum. Molec. Genet. 2: 1291-1293, 1993. 3. Bruno, C., Lofberg, M., Tamburino, L., Jankala, H., Hadjigeorgiou, G. M., Andreu, A. L., Shanske, S., Somer, H., DiMauro, S. Molecular characterization of McArdle's disease in two large Finnish families. J. Neurol. Sci. 165: 121-125, 1999.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Calculated Molecular Weight

97 kDa

Applications Notes

6

Gene Name Synonym

Glycogen phosphorylase, muscle associated

Protein Name

Glycogen phosphorylase, muscle form

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

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