Novus Biologicals products are now on bio-techne.com

LYAG/GAA Antibody (2489B) [Unconjugated]

Images

 
Western blot shows lysates of T47D human breast cancer cell line and human peripheral blood mononuclear cells (PBMC). PVDF membrane was probed with 1 µg/mL of Rabbit Anti-Human Lysosomal a-Glucosidase Monoclonal ...read more
Lysosomal alpha ‑Glucosidase was detected in immersion fixed paraffin-embedded sections of human kidney using Rabbit Anti-Human Lysosomal alpha ‑Glucosidase Monoclonal Antibody (Catalog # MAB83291) at 3 ...read more

Product Details

Summary
Reactivity HuSpecies Glossary
Applications WB, IHC
Clone
2489B
Clonality
Monoclonal
Host
Rabbit
Conjugate
Unconjugated

Order Details

View Available Formulations
Catalog# & Formulation Size Price
Novus Biologicals is part of Bio-Techne

Shop this product on bio-techne.com

LYAG/GAA Antibody (2489B) [Unconjugated] Summary

Additional Information
Recombinant Monoclonal Antibody.
Immunogen
Human embryonic kidney cell HEK293-derived human Lysosomal alpha -Glucosidase
Ala70-Cys952
Accession # P10253
Specificity
Detects human Lysosomal alpha -Glucosidase in direct ELISAs. Detects only 76Kda cleaved band.
Source
N/A
Isotype
IgG
Clonality
Monoclonal
Host
Rabbit
Purity Statement
Protein A or G purified from cell culture supernatant
Innovator's Reward
Test in a species/application not listed above to receive a full credit towards a future purchase.

Applications/Dilutions

Dilutions
  • Immunohistochemistry 3-25 ug/mL
  • Western Blot 1 ug/mL

Packaging, Storage & Formulations

Storage
Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
  • 12 months from date of receipt, -20 to -70 °C as supplied.
  • 1 month, 2 to 8 °C under sterile conditions after reconstitution.
  • 6 months, -20 to -70 °C under sterile conditions after reconstitution.
Buffer
Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied either lyophilized or as a 0.2 µm filtered solution in PBS.
Reconstitution Instructions
Reconstitute at 0.5 mg/mL in sterile PBS.

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for LYAG/GAA Antibody (2489B) [Unconjugated]

  • Acid alpha-Glucosidase
  • Acid Maltase
  • Aglucosidase alfa
  • EC 3.2.1.20
  • GAA
  • glucosidase, alpha; acid
  • LYAG
  • Lysosomal alphaGlucosidase
  • Lysosomal alpha-Glucosidase

Background

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).
  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.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Customers Who Viewed This Item Also Viewed...

NBP2-01743
Species: Hu
Applications: Flow, ICC/IF, IHC, IHC-P, WB
NBP3-38293
Species: Hu, Mu, Rt
Applications: ELISA, ICC/IF, IHC, IHC-P, WB
NBP1-30873
Species: Hu, Mu, Rt
Applications: ChIP, ICC/IF, IHC, IHC-P, WB
NBP1-88866
Species: Hu
Applications: IHC, IHC-P
NBP2-13075
Species: Ba, Hu, Pm, Mu, Rt
Applications: DB, ELISA, IB, ICC/IF, IHC-FrFl, IHC, IHC-Fr, IHC-P, IP, WB
H00008086-M02
Species: Hu, Mu, Rt
Applications: ELISA, IHC, IHC-P, WB
NBP2-58917
Species: Hu
Applications: IHC, IHC-P, WB
NBP1-76544
Species: Hu, Mu, Rt
Applications: ELISA, ICC/IF, IHC, IHC-P, Simple Western, WB
AF2009
Species: Hu
Applications: ICC, IHC
NBP1-89766
Species: Hu, Mu, Rt
Applications: ICC/IF, IHC, IHC-P, WB
MAB7616
Species: Hu
Applications: CyTOF-ready, Flow, WB
MAB1417
Species: Bv, Hu, Mu
Applications: CyTOF-ready, ICC, IHC, ICFlow
NBP1-87094
Species: Hu
Applications: ICC/IF, IHC, IHC-P
NBP1-87377
Species: Hu
Applications: ICC/IF, IHC, IHC-P, WB
NBP1-89785
Species: Hu
Applications: ICC/IF, IHC, IHC-P, WB
NBP2-22217
Species: Ch, Hu, Mu, Rt(-)
Applications: CyTOF-ready, ELISA, Flow, IB, ICC/IF, IHC, IHC-Fr, IHC-P, KO, Simple Western, WB

Publications for LYAG/GAA Antibody (MAB83291) (0)

There are no publications for LYAG/GAA Antibody (MAB83291).
By submitting your publication information earn gift cards and discounts for future purchases.

Reviews for LYAG/GAA Antibody (MAB83291) (0)

There are no reviews for LYAG/GAA Antibody (MAB83291). By submitting a review you will receive an Amazon e-Gift Card or Novus Product Discount.
  • Review with no image -- $10/€7/£6/$10 CAD/¥70 Yuan/¥1110 Yen
  • Review with an image -- $25/€18/£15/$25 CAD/¥150 Yuan/¥2500 Yen

Product General Protocols

Find general support by application which include: protocols, troubleshooting, illustrated assays, videos and webinars.

Video Protocols

WB Video Protocol

FAQs for LYAG/GAA Antibody (MAB83291) (0)

There are no specific FAQs related to this product. Read our general customer & technical service FAQs.
Read our latest blog and use the new citation tool on bio-techne.com

Customers Who Bought This Also Bought

Contact Information

Product PDFs

Calculators

Concentration Calculator

The concentration calculator allows you to quickly calculate the volume, mass or concentration of your vial. Simply enter your mass, volume, or concentration values for your reagent and the calculator will determine the rest.

=
÷

Review this Product

Be the first to review our LYAG/GAA Antibody (2489B) [Unconjugated] and receive a gift card or discount.

Bioinformatics

Uniprot