Human Lysosomal alpha -Glucosidase Antibody

Recombinant Monoclonal Antibody
Catalog # Availability Size / Price Qty
MAB8329-100
MAB8329-SP
Human Lysosomal  alpha -Glucosidase Antibody in Western Blot
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Human Lysosomal alpha -Glucosidase Antibody Summary

Species Reactivity
Human
Specificity
Detects human Lysosomal alpha -Glucosidase protein in direct ELISAs.
Source
Recombinant Monoclonal Rabbit IgG Clone # 2489C
Purification
Protein A or G purified from cell culture supernatant
Immunogen
Human embryonic kidney cell, HEK293-derived human Lysosomal alpha -Glucosidase protein
Ala70-Cys952
Accession # P10253
Formulation
Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied as a 0.2 µm filtered solution in PBS.
Label
Unconjugated

Applications

Recommended Concentration
Sample
Western Blot
1 µg/mL
See below
Immunohistochemistry
3-25 µg/mL
See below

Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.

Data Examples

Western Blot Detection  of Human Lysosomal  alpha ‑Glucosidase by Western Blot. View Larger

Detection of Human Lysosomal alpha ‑Glucosidase by Western Blot. Western blot shows lysates of human placenta tissue, T47D human breast cancer cell line, and MCF‑7 human breast cancer cell line. PVDF membrane was probed with 1 µg/mL of Rabbit Anti-Human Lysosomal alpha ‑Glucosidase Monoclonal Antibody (Catalog # MAB8329) followed by HRP-conjugated Anti-Rabbit IgG Secondary Antibody (Catalog # HAF008). Specific bands were detected for Lysosomal alpha ‑Glucosidase at approximately 72 kDa, 75 kDa, and 105 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1.

Immunohistochemistry Lysosomal  alpha-Glucosidase in Human Kidney. View Larger

Lysosomal alpha-Glucosidase in Human Kidney. Lysosomal alpha ‑Glucosidase was detected in immersion fixed paraffin-embedded sections of human kidney using Rabbit Anti-Human Lysosomal alpha ‑Glucosidase Monoclonal Antibody (Catalog # MAB8329) at 3 µg/mL for 1 hour at room temperature followed by incubation with the Anti-Rabbit IgG VisUCyte™ HRP Polymer Antibody (Catalog # VC003). Before incubation with the primary antibody, tissue was subjected to heat-induced epitope retrieval using Antigen Retrieval Reagent-Basic (Catalog # CTS013). Tissue was stained using DAB (brown) and counterstained with hematoxylin (blue). Specific staining was localized to cytoplasm in convoluted tubules. View our protocol for IHC Staining with VisUCyte HRP Polymer Detection Reagents.

Reconstitution Calculator

Reconstitution Calculator

The reconstitution calculator allows you to quickly calculate the volume of a reagent to reconstitute your vial. Simply enter the mass of reagent and the target concentration and the calculator will determine the rest.

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Preparation and Storage

Reconstitution
Reconstitute at 0.5 mg/mL in sterile PBS.
Reconstitution Buffer Available
Reconstitution Buffer 1 (PBS)
Catalog #
Availability
Size / Price
Qty
RB01
Shipping
The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °C
Stability & 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.

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
Entrez Gene IDs
2548 (Human); 14387 (Mouse); 367562 (Rat)
Alternate Names
Acid alpha-Glucosidase; Acid Maltase; Aglucosidase alfa; EC 3.2.1.20; GAA; glucosidase, alpha; acid; LYAG; Lysosomal alphaGlucosidase; Lysosomal alpha-Glucosidase

Product Datasheets

FAQs

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Cell and Tissue Staining Kits

Immunohistochemistry Reagents

Isotype Controls

Normal Rabbit IgG Control

Ctrl,CyTOF-ready

Reconstitution Buffers

Secondary Antibodies

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