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

Chromosome: 11; NC_000077.6 (119267967..119285698). Location: 11 D-E; 11 83.35 cM

E Coli

> 95%

Supplied as lyophilized form in PBS,pH7.4, containing 5% sucrose, 0.01% sarcosyl.

Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months.
Stability Test: The thermal stability is described by the loss rate of the targetprotein. The loss rate was determined by accelerated thermal degradation test,that is, incubate the protein at 37 degree C for 48h, and no obvious degradation andprecipitation were observed. (Referring from China Biological Products Standard,which was calculated by the Arrhenius equation.) The loss of this protein is lessthan 5% within the expiration date under appropriate storage condition.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of GaA recombinant protein 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.

About the Marker: Effective Size Range: 10kDa to 70kDa.
Protein bands: 10kDa, 14kDa, 18kDa, 22kDa, 26kDa, 33kDa, 44kDa and70kDa.
Double intensity bands: The 26kDa, 18kDa, 10kDa bands are at doubleintensity to make location and size approximation of proteins of interestquick and easy.
Ready-to-use: No need to heat, dilute or add reducing agents before use.

SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)

22.9kDa

glucosidase, alpha, acid

lysosomal alpha-glucosidase; acid maltase

Lysosomal alpha-glucosidase

LYAG_MOUSE

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: Contractile; EC 3.2.1.20; Carbohydrate Metabolism - galactose; Hydrolase; Carbohydrate Metabolism - starch and sucrose

Cellular Component: membrane; lysosomal membrane; lysosome

Molecular Function: hydrolase activity; hydrolase activity, acting on glycosyl bonds; alpha-glucosidase activity; maltase activity; carbohydrate binding; catalytic activity; hydrolase activity, hydrolyzing O-glycosyl compounds

Biological Process: heart morphogenesis; glycogen metabolic process; tissue development; striated muscle contraction; glycogen catabolic process; metabolic process; vacuolar sequestering; locomotory behavior; muscle maintenance; neuromuscular process controlling posture; lysosome organization and biogenesis; diaphragm contraction; carbohydrate metabolic process; neuromuscular process controlling balance; regulation of the force of heart contraction; cardiac muscle contraction

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While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

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