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]
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).
-
Hoefsloot L.H. et al. (1988) EMBO J. 7:1697.
- Wan, L. et al. (2008) J. Neurol. 255:831.
- Fukuda, T. et al. (2007) Curr. Neurol. Neurosci. Rep. 7:71.
- Van Gelder, C.M. et al. (2014) J Inherit Metab Dis. In press.
- 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.
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