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Anti-GAA Antibody Picoband® Fluoro594 Conjugated

Product Specifications

Background

Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. 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.

Synonyms

Lysosomal alpha-glucosidase;3.2.1.20; Acid maltase; Aglucosidase alfa;76 kDa lysosomal alpha-glucosidase;70 kDa lysosomal alpha-glucosidase; GAA

Gene Name

GAA

Gene ID

2548

UniProt

P10253

Host

Rabbit

Reactivity

Human

Cross Reactivity

No cross-reactivity with other proteins

Immunogen

A synthetic peptide corresponding to a sequence in the middle region of human GAA, different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids.

Clonality

Polyclonal

Tissue Specificity

Most abundant in heart, brain, liver, skeletal muscle and testis but absent in thymus and peripheral blood leukocytes.

Applications

Flow Cytometry

Field of Research

Cancer, Energy Metabolism, Energy Transfer Pathways, Metabolic Signaling Pathways, Metabolism, Pathways and Processes, Signal Transduction

Purification

Immunogen affinity purified.

Form

Liquid

Function

Essential for the degradation of glygogen to glucose in lysosomes.

References & Citations

1. Entrez Gene: GAA glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II) . 2. Donald J. Voet; Judith G. Voet; Charlotte W. Pratt (2008) . Additional Pathways in Carbohydrate Metabolism. Principles of Biochemistry, Third edition. Wiley. p. 538. 3. Reuser AJ, Kroos MA, Hermans MM, et al. (1995) . Glycogenosis type II (acid maltase deficiency) .. Muscle Nerve. 3: S61–9.

Storage Conditions

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

Calculated Molecular Weight

105324 MW

Specificity

No cross reactivity with other proteins.

Applications Notes

6

Gene Name Synonym

Lysosomal alpha-glucosidase

Subcellular Location

Lysosome . Lysosome membrane .

Protein Name

Lysosomal alpha-glucosidase

Isotype

Rabbit IgG

Contents

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

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