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

Polyclonal

Rabbit

Affinity Purified

Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

For short term use, store at 2-8 degree C up to 1 week. For long term storage, store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-GAA antibody 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.

This is a rabbit polyclonal antibody against GAA. It was validated on Western Blot using a cell lysate as a positive control.

Target Description: GAA is 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. 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.

Polyclonal; Signal Proteins; Membrane Protein; Drugs and Drug Metabolism; Neuroscience; Disease Related;

Immunohistochemistry (IHC), Western Blot (WB)

Rabbit Anti-GAA Antibody
Formalin Fixed Paraffin Embedded Tissue: Human Lung Tissue
Observed Staining: Cytoplasmic in alveolar type I cells
Primary Antibody Concentration: 1:100
Other Working Concentrations: 1/600
Secondary Antibody: Donkey anti-Rabbit-Cy3
Secondary Antibody Concentration: 1:200
Magnification: 20X
Exposure Time: 0.5 - 2.0 sec

WB Suggested Anti-GAA Antibody Titration: 0.2-1 ug/ml
ELISA Titer: 1:62500
Positive Control: MCF7 cell lysateGAA is supported by BioGPS gene expression data to be expressed in MCF7

98kDa

glucosidase alpha, acid

lysosomal alpha-glucosidase

Lysosomal alpha-glucosidase

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]

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

Chromosomal Location of Human Ortholog: 17q25.3

Cellular Component: lysosomal lumen; lysosomal membrane; lysosome; membrane

Molecular Function: alpha-glucosidase activity; oligo-1,6-glucosidase activity

Biological Process: cardiac muscle contraction; diaphragm contraction; glucose metabolic process; glycogen catabolic process; lysosome organization and biogenesis; maltose metabolic process; sucrose metabolic process; vacuolar sequestering

Disease: Glycogen Storage Disease Ii

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