Human Lysosomal alpha ‑Glucosidase Alexa Fluor™ Plus 680‑conjugated Antibody

R&D Systems | Catalog # FAB83291AFP680

R&D Systems

Key Product Details

Species Reactivity

Human

Applications

Immunohistochemistry, Western Blot

Label

Alexa Fluor Plus 680 (Excitation = 687 nm, Emission = 704 nm)

Antibody Source

Recombinant Monoclonal Rabbit IgG Clone # 2489B
View all Lysosomal alpha-Glucosidase Primary Antibodies »

Product Specifications

Specificity

Detects human Lysosomal alpha -Glucosidase in direct ELISAs. Detects only 76Kda cleaved band.

Clonality

Monoclonal

Host

Rabbit

Isotype

IgG

Applications

Application
Recommended Usage

Immunohistochemistry

Optimal dilution of this antibody should be experimentally determined.

Western Blot

Optimal dilution of this antibody should be experimentally determined.

Formulation, Preparation, and Storage

Formulation

Supplied 0.2 mg/mL in a saline solution containing BSA and Sodium Azide.

Shipping

The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied

Background: Lysosomal alpha-Glucosidase

Acid alpha-glucosidase (GAA) is an enzyme that is essential in the degradation of glycogen to glucose in the lysosome (1). Defects in GAA are the cause of glycogen storage disease II, also known as Pompe's disease, which is a rare autosomal recessive metabolic disorder that damages muscle and nerve cells throughout the body, primarily due to the accumulation of glycogen in the lysosome (2). Pompe disease occurs in babies, children, and adults who inherit a defective GAA gene and affects an estimated 5,000 to 10,000 people worldwide (3). Enzyme replacement therapy (ERT) is used to treat patients with this disease (4, 5).

References

  1. Hoefsloot L.H. et al. (1988) EMBO J. 7:1697.
  2. Wan, L. et al. (2008) J. Neurol. 255:831.
  3. Fukuda, T. et al. (2007) Curr. Neurol. Neurosci. Rep. 7:71.
  4. Van Gelder, C.M. et al. (2014) J Inherit Metab Dis. In press.
  5. Toscano, A. and Schoser, B. (2013) J. Neurol. 260:951.

Long Name

Glucosidase, Alpha; Acid

Alternate Names

Acid alpha-Glucosidase, Acid Maltase, GAA, LYAG, Lysosomal alphaGlucosidase

Entrez Gene IDs

2548 (Human); 14387 (Mouse); 367562 (Rat); 102141245 (Cynomolgus Monkey)

Gene Symbol

GAA

UniProt

P10253

Additional Lysosomal alpha-Glucosidase Products

Product Documents

Certificate of Analysis

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Note: Certificate of Analysis not available for kit components.

Product Specific Notices


This product is provided under an intellectual property license from Life Technologies Corporation. The transfer of this product is conditioned on the buyer using the purchased product solely in research conducted by the buyer, excluding contract research or any fee for service research, and the buyer must not (1) use this product or its components for (a) diagnostic, therapeutic or prophylactic purposes; (b) testing, analysis or screening services, or information in return for compensation on a per-test basis; or (c) manufacturing or quality assurance or quality control, and/or (2) sell or transfer this product or its components for resale, whether or not resold for use in research. For information on purchasing a license to this product for purposes other than as described above, contact Life Technologies Corporation, 5781 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@thermofisher.com.

For research use only

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Protocols

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FAQs

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Associated Pathways

VEGF - VEGF R2 Signaling Pathways
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