CINXE.COM
Recombinant Human Lysosomal alpha-Glucosidase/GAA, CF 8329-GH-025: R&D Systems
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id="content_column"> <div class="ds_title_table"> <h1 class="ds_title">Recombinant Human Lysosomal alpha-Glucosidase/GAA, CF </h1> <div class="dsSprite_rnd ds_brand_logo ds_rnd_logo"></div> </div> <div class="product_meta"> <span class="category_number">Catalog #: 8329-GH</span> <span class="reviews review_stars_5"> <a href="#product-reviews" id="product_reviews" class="jump_to" data-tab="6" data-tabname="product-reviews"> 4 Reviews </a> </span> <a href="/reviews/8329-GH" rel="nofollow"><span class="submit_review"><button class="btn btn-quaternary">Submit a Review</button></span></a> <span class="datasheet_link"> <a href="#product-datasheets">Datasheet / COA / SDS</a> </span> </div> <!--<div class="formulations_row row"> <div class="formulations_wrapper col-xs-12"> </div> </div>--> <div class="product_wrapper row"> <div class="product_ordering_wrapper col-xs-12 col-md-8 col-md-push-4"> <div class="product_ordering"> <form action="/products/recombinant-human-lysosomal-alpha-glucosidase-gaa-cf_8329-gh" method="post" id="atc-datasheet-form" accept-charset="UTF-8"><div><div id="atc_datasheet_block"> <!-- Carrier Free Section --> <!-- Single Use Lyophilized Section --> <!-- Liquid Section --> <!-- Regular Products Section --> <table class="add_to_cart"> <thead> <tr> <th class="ds_catalog">Catalog #</th> <th class="ds_availability_column mobile-hide">Availability</th> <th class="ds_size_price_column">Size / Price</th> <th class="ds_quantity_column">Qty</th> </tr> </thead> <tbody> <tr class="et_atc_product" data-sku="8329-GH-025"> <td class="dat_catalog_column">8329-GH-025</td> <td class="et_atc_results" colspan="2"></td> <td class="ds_quantity ds_quantity_column"><div class="form-item form-item-catnum-8329-gh-025 form-type-textfield form-group"><input class="atc_quantity atc_hidden atc_qty form-control form-text" placeholder="0" style="display:none;" type="text" id="edit-catnum-8329-gh-025" name="catnum-8329-GH-025" value="" size="5" maxlength="3" /></div></td> </tr> </tbody> </table> <div class="ds_atc_pre_buttons"></div> <div class="ds_atc_buttons"> <a href="/quote/instruments/ella-automated-immunoassay-system" class="btn btn-secondary atc_request_quote_simple_plex_assay" style="display:none" rel="nofollow">Request a Quote</a> <a href="/services/bulk-quote-request?catnum=8329-GH&product=Recombinant Human Lysosomal alpha-Glucosidase/GAA, CF" class="btn btn-secondary atc_hidden bulk_btn" style="display:none;" rel="nofollow" title="Competitive discounts on products purchased in bulk quantities">Bulk Orders</a> <button class="btn btn-primary atc_hidden atc_btn form-submit icon-before" style="display:none;" title="Enter quantities above and click to add to cart" type="submit" id="edit-add-to-cart" name="add_to_cart_btn" value="Add to Cart"><span class="icon glyphicon glyphicon-plus" aria-hidden="true"></span> Add to Cart</button> </div> <div 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No. 8329-GH - Recombinant Human Lysosomal alpha-Glucosidase/GAA, CF" href="https://resources.rndsystems.com/images/datasheets/8329-gh_recombinant-human-lysosomal-alpha-glucosidase-gaa-cf-enzyme-activity-622023983.jpg"> <img fetchpriority="high" src="https://resources.rndsystems.com/images/datasheets/8329-gh_recombinant-human-lysosomal-alpha-glucosidase-gaa-cf-enzyme-activity-622023983.jpg" class="img-fluid" alt="Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein Enzyme Activity Diagram." title="Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein Enzyme Activity Diagram."> </a> <div class="search_image_other_count"> <span class="search_more_images">3 Images</span> </div> </div> </div> <div id="extra_images overview-images" class="hidden"> <a class="ds-image" data-toggle="lightbox" data-gallery="overview-images" data-footer="Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein Enzyme Activity." data-title="Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein Enzyme Activity." title="Cat. No. 8329-GH - Recombinant Human Lysosomal alpha-Glucosidase/GAA, CF" onClick="event.preventDefault();" href="https://resources.rndsystems.com/images/datasheets/8329-gh_recombinant-human-lysosomal-alpha-glucosidase-gaa-cf-enzyme-activity-6220239264.jpg"> <img src="https://resources.rndsystems.com/images/datasheets/8329-gh_recombinant-human-lysosomal-alpha-glucosidase-gaa-cf-enzyme-activity-6220239264.jpg" class="img-fluid" loading="lazy" alt="Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein Enzyme Activity." title="Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein Enzyme Activity."> </a> <a class="ds-image" data-toggle="lightbox" data-gallery="overview-images" data-footer="Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein SDS-PAGE." data-title="Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein SDS-PAGE." title="Cat. No. 8329-GH - Recombinant Human Lysosomal alpha-Glucosidase/GAA, CF" onClick="event.preventDefault();" href="https://resources.rndsystems.com/images/datasheets/8329-gh_recombinant-human-lysosomal-alpha-glucosidase-gaa-cf-sds-page-62202393418.jpg"> <img src="https://resources.rndsystems.com/images/datasheets/8329-gh_recombinant-human-lysosomal-alpha-glucosidase-gaa-cf-sds-page-62202393418.jpg" class="img-fluid" loading="lazy" alt="Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein SDS-PAGE." title="Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein SDS-PAGE."> </a> </div> </div> <div class="product_info_wrapper"> </div> <div id="view-more-target" class="datasheet_section_element"> <a href="/target/lysosomal-alpha-glucosidase?category=Proteins and Enzymes" class="btn btn-secondary">All Lysosomal alpha-Glucosidase Proteins and Enzymes</a> </div> </div> <div class="tabbed_content" id="rnd_protein"> <div id="ds_tabs" class="tab_menu"> <div class="tab tab_1 tab_open" data-tab="1" data-tabname="product-details" id="dsTabs_product-details"> <span>Product Details</span> </div> <div class="tab tab_4" data-tab="4" data-tabname="faqs" id="dsTabs_faqs"> <span>FAQs</span> </div> <div class="tab tab_6" data-tab="6" data-tabname="product-reviews" id="dsTabs_product-reviews"> <span> Reviews (4) </span> </div> </div> <div class="tab_content tab_1 tab_open" data-tab="1" data-tabname="product-details" id="product-details"> <div class="datasheet_section_element"> <a class="btn btn-quaternary scroll_to anchor-link" href="#ds_summary" id="product-anchor-ds_summary">Summary</a> <a class="btn btn-quaternary scroll_to anchor-link" href="#ds_datasheets" id="product-anchor-ds_datasheets">Product Datasheets</a> <a class="btn btn-quaternary scroll_to anchor-link" href="#ds_carrier_free" id="product-anchor-ds_carrier_free">Carrier Free</a> <a class="btn btn-quaternary scroll_to anchor-link" href="#ds_data_examples" id="product-anchor-ds_data_examples">Data Images</a> <a class="btn btn-quaternary scroll_to anchor-link" href="#ds_calculator" id="product-anchor-ds_calculator">Reconstitution Calculator</a> <a class="btn btn-quaternary scroll_to anchor-link" href="#ds_background" id="product-anchor-ds_background">Background</a> <a class="btn btn-quaternary scroll_to anchor-link" href="#ds_related_research_areas" id="product-anchor-ds_related_research_areas">Related Research Areas</a> </div> <div id="ds_summary" class="datasheet_section"> <h2>Recombinant Human Lysosomal alpha-Glucosidase/GAA, CF Summary</h2> <div class="what-statement-container"> </div> <div class="why-statement-container"> </div> <h4 class="mt-3">Product Specifications</h4> <div class="ds_table summary_table"> <div class="ds_table_body"> <div class="ds_table_row"> <div class="ds_table_cell summary_table_name">Purity</div> <div class="ds_table_cell summary_table_data">>95%, by SDS-PAGE under reducing conditions and visualized by Colloidal Coomassie® Blue stain at 5 μg per lane.</div> </div> <div class="ds_table_row"> <div class="ds_table_cell summary_table_name">Endotoxin Level</div> <div class="ds_table_cell summary_table_data"><0.10 EU per 1 μg of the protein by the LAL method.</div> </div> <div class="ds_table_row"> <div class="ds_table_cell summary_table_name">Activity</div> <div class="ds_table_cell summary_table_data">Measured by its ability to release glucose from starch. The specific activity is >7,500 pmol/min/μg, as measured under the described conditions.</div> </div> <div class="ds_table_row"> <div class="ds_table_cell summary_table_name">Source</div> <div class="ds_table_cell summary_table_data">Human embryonic kidney cell, HEK293-derived human Lysosomal alpha-Glucosidase protein<br />Ala70-Cys952, with an N-terminal 6-His tag</div> </div> <div class="ds_table_row"> <div class="ds_table_cell summary_table_name">Accession #</div> <div class="ds_table_cell summary_table_data"><a href="http://www.ncbi.nlm.nih.gov/protein/P10253" target="_blank" rel="noreferrer nofollow">P10253</a></div> </div> <div class="ds_table_row"> <div class="ds_table_cell summary_table_name">N-terminal Sequence<br />Analysis</div> <div class="ds_table_cell summary_table_data">His</div> </div> <div class="ds_table_row"> <div class="ds_table_cell summary_table_name">Predicted Molecular Mass</div> <div class="ds_table_cell summary_table_data">99 kDa</div> </div> <div class="ds_table_row"> <div class="ds_table_cell summary_table_name">SDS-PAGE</div> <div class="ds_table_cell summary_table_data">95-105 kDa, reducing conditions</div> </div> </div> </div> </div> <div id="customer_also_purchased_block" class="loader" data-catalogId="8329-GH"></div> <div class="datasheet_section" id="product-datasheets"> <h2 id="ds_datasheets">Product Datasheets</h2> <div class="product_datasheets"> <span class="sds-message hide"> <p class="sds-label">You must select a language.</p> <span class="close_sds_message">x</span> </span> <h2>8329-GH </h2> <div class="ds_document"> <a id="cfpdf" href="//resources.rndsystems.com/pdfs/datasheets/8329-gh.pdf?v=20241112" class="datasheets-link " target="_blank" rel="noreferrer nofollow">Product Datasheet</a> </div> <div class="ds_document"> <div class="ds_pdfData"><a class="datasheets-link" href="https://www.rndsystems.com/cofas/recombinant-human-lysosomal-alpha-glucosidase-gaa-cf_8329-gh">COA</a> </div> </div> <div class="ds_document sds_link"> <a class="sds_non_cf datasheets-link non_cf_download_link" target="_blank" rel="noreferrer" data-path="//aero.bio-techne.com/sds/pdf/8329-gh?v=20241112">SDS</a> </div> </div> </div> <div id="ds_carrier_free" class="datasheet_section"> <h2>Carrier Free</h2> <div class="highlight">What does CF mean?</div> <p>CF stands for Carrier Free (CF). We typically add Bovine Serum Albumin (BSA) as a carrier protein to our recombinant proteins. Adding a carrier protein enhances protein stability, increases shelf-life, and allows the recombinant protein to be stored at a more dilute concentration. The carrier free version does not contain BSA. </p> <div class="highlight">What formulation is right for me?</div> <p>In general, we advise purchasing the recombinant protein with BSA for use in cell or tissue culture, or as an ELISA standard. In contrast, the carrier free protein is recommended for applications, in which the presence of BSA could interfere. </p> <div class="cf_tables row"> <div class="cf_table_1 col-xs-12"> <h4 class="cf_table_name">8329-GH</h4> <table class="ds_table_html carrier_free_table"> <tbody> <tr> <td class="cf_key_name">Formulation</td> <td>Supplied as a 0.2 μm filtered solution in Tris, NaCl and Glycerol.</td> </tr> <tr> <td class="cf_key_name">Shipping</td> <td>The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.</td> </tr> <tr> <td class="cf_key_name">Stability & Storage:</td> <td><b>Use a manual defrost freezer and avoid repeated freeze-thaw cycles. </b><UL><LI>12 months from date of receipt, -20 to -70 °C as supplied.</LI><LI>6 months, -20 to -70 °C under sterile conditions after opening.</LI></UL></td> </tr> </tbody> </table> </div> </div> </div> <div id="pd_assay_procedure" class="datasheet_section"> <h2>Assay Procedure</h2> <div><strong>Materials</strong></div> <div><UL> <LI>Assay Buffer: 0.1 M Sodium Acetate, pH 4.5</LI> <LI>Recombinant Human Lysosomal alpha -Glucosidase (rhGAA) (Catalog # 8329-GH)</LI> <LI>Stop Solution: 4.4 mM Dinitrosalicylic Acid, 1 M Potassium Tartrate, 0.4 M Sodium Hydroxide in deionized water</LI> <LI>Standard: Maltose (Sigma, Catalog # M5885), 20 mM stock in deionized water</LI> <LI>Substrate: Starch from potato (Sigma, Catalog # 85642), 2% (w/v) stock in deionized water</LI> <LI>96-well Clear Plate (Catalog # <A class=NoLineLink href="http://www.rndsystems.com/product_results.aspx?k=DY990">DY990</A>)</LI> <LI>Plate Reader (Model: SpectraMax Plus by Molecular Devices) or equivalent</LI></UL> <OL> <LI>Dilute 20 mM Maltose standard by adding 200 µL of the 20 mM Maltose Standard to 600 µL of Assay Buffer for a 5 mM stock. This is the first point of the standard curve.</LI> <LI>Prepare the standard curve by performing five one-half serial dilutions of the 5 mM Maltose stock in Assay Buffer. Make sure there are 400 μL in each tube for each point of the curve (remove 400 μL from the last point of the curve). Prepare one tube with only 400 μL of Assay Buffer for the curve blank. The standard curve has a range of 19.5 to 625 nmol per well.</LI> <LI>Dilute rhGAA to 32 ng/μL in Assay Buffer.</LI> <LI>Dilute 2% starch to 1% in Assay Buffer by combining equal volumes of 2% starch and Assay Buffer.</LI> <LI>Prepare reactions by combining 20 μL of diluted rhGAA with 380 μL of 1% starch (step 4). Include a control by combining 20 μL of Assay Buffer with 380 μL of 1% starch.</LI> <LI>Vortex, spin, and then incubate reactions, control and standard curve at 37 °C for 1 hour.</LI> <LI>Add 400 uL of Stop Solution to all vials, including standard curve.</LI> <LI>Heat all vials at 95-100 °C for 6 minutes. Then, cool on ice. Tip: Use lid-locks on vials when heating.</LI> <LI>Load 250 µL of each dilution of the standard curve, reactions and controls to empty wells in triplicate in plate.</LI> <LI>Read plate at 546 nm (absorbance) in endpoint mode.</LI> <LI>Calculate specific activity:</LI></OL> <TABLE> <TBODY> <TR> <TD rowSpan=2 align=right> <P> Specific Activity (pmol/min/µg) =</P></TD> <TD style="BORDER-BOTTOM: black 1pt solid; PADDING-BOTTOM: 2px" align=middle>Adjusted glucose produced* (nmol) x (1000 pmol/nmol)</TD></TR> <TR> <TD style="BORDER-BOTTOM: black 0pt solid" align=middle>Incubation time (min) x amount of enzyme (µg)</TD></TR></TBODY></TABLE> <P> *Derived from the maltose standard curve using linear or 4-parameter fitting and adjusted for curve blank.</P> Per Well: <UL> <LI>rhGAA: 0.200 µg</LI> <LI>Starch: 0.475%</LI></UL></div> </div> <div id="ds_scientific_data" class="datasheet_section"> <div id="ds_data_examples"> <h2>Scientific Data</h2> <div id="data_examples"> <div class="row data_ex_row"> <a id="ds_image_0" class="pageAnchor"></a> <div class="data_ex_image_wrapper col-xs-12 col-sm-5 col-md-4"> <div class="data_ex_image"> <span class="data_ex_image_label"> Enzyme Activity </span> <a class="ds-image overview-images data_ex_image_link" data-toggle="lightbox" data-gallery="overview-images-0" data-title="Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein Enzyme Activity Diagram." data-footer="" title="Cat. No. - " href="https://resources.rndsystems.com/images/datasheets/8329-gh_recombinant-human-lysosomal-alpha-glucosidase-gaa-cf-enzyme-activity-622023983.jpg"> <img loading="lazy" src="https://resources.rndsystems.com/images/datasheets/8329-gh_recombinant-human-lysosomal-alpha-glucosidase-gaa-cf-enzyme-activity-622023983.jpg" class="img-fluid" alt="" title=""> <span class="data_ex_image_larger">View Larger</span> </a> </div> </div> <div class="data_ex_description col-xs-12 col-sm-7 col-md-8"> <p> Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein, CF (Catalog # 8329-GH) hydrolyses both alpha-1,4- and alpha-1,6-glucosidic linkages on glycogen to release terminal glucose. </p> </div> </div> <div class="row data_ex_row"> <a id="ds_image_1" class="pageAnchor"></a> <div class="data_ex_image_wrapper col-xs-12 col-sm-5 col-md-4"> <div class="data_ex_image"> <span class="data_ex_image_label"> Enzyme Activity </span> <a class="ds-image overview-images data_ex_image_link" data-toggle="lightbox" data-gallery="overview-images-1" data-title="Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein Enzyme Activity." data-footer="" title="Cat. No. - " href="https://resources.rndsystems.com/images/datasheets/8329-gh_recombinant-human-lysosomal-alpha-glucosidase-gaa-cf-enzyme-activity-6220239264.jpg"> <img loading="lazy" src="https://resources.rndsystems.com/images/datasheets/8329-gh_recombinant-human-lysosomal-alpha-glucosidase-gaa-cf-enzyme-activity-6220239264.jpg" class="img-fluid" alt="" title=""> <span class="data_ex_image_larger">View Larger</span> </a> </div> </div> <div class="data_ex_description col-xs-12 col-sm-7 col-md-8"> <p> Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein, CF (Catalog # 8329-GH) is measured by its ability to release glucose from starch. </p> </div> </div> <div class="row data_ex_row"> <a id="ds_image_2" class="pageAnchor"></a> <div class="data_ex_image_wrapper col-xs-12 col-sm-5 col-md-4"> <div class="data_ex_image"> <span class="data_ex_image_label"> SDS-PAGE </span> <a class="ds-image overview-images data_ex_image_link" data-toggle="lightbox" data-gallery="overview-images-2" data-title="Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein SDS-PAGE." data-footer="" title="Cat. No. - " href="https://resources.rndsystems.com/images/datasheets/8329-gh_recombinant-human-lysosomal-alpha-glucosidase-gaa-cf-sds-page-62202393418.jpg"> <img loading="lazy" src="https://resources.rndsystems.com/images/datasheets/8329-gh_recombinant-human-lysosomal-alpha-glucosidase-gaa-cf-sds-page-62202393418.jpg" class="img-fluid" alt="" title=""> <span class="data_ex_image_larger">View Larger</span> </a> </div> </div> <div class="data_ex_description col-xs-12 col-sm-7 col-md-8"> <p> 2 μg/lane of Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein (Catalog # 8329-GH) was resolved with SDS-PAGE under reducing (R) and non-reducing (NR) conditions and visualized by Coomassie® Blue staining, showing bands at 95-105 kDa. </p> </div> </div> </div> </div> </div> <div id="ds_calculator" class="datasheet_section"> <div id="btn_calculator_wrapper"><div class="datasheet_calculator_toggle btn btn-secondary" id="btn_reconstitution">Reconstitution Calculator</div></div><div id="datasheet_calculators"><div id="btn_reconstitution_calc" class="datasheet_calculator"><h2>Reconstitution Calculator</h2><div id="reconstitution_calculator" class="calculator"> <form id="recCalc"> <div class="reconstitution_calculator_text" id="reconstitution_calculator_instructions"> <p>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.</p> </div> <div class="calc_section"> <label for="reconstitution_calculator_volume" class="calculator_label">Volume (to add to vial)</label> <input id="reconstitution_calculator_volume" name="reconstitution_calculator_volume" type="text" maxlength="16" placeholder="volume" class="calculator_input calculator_text"/> <span class="select_wrapper"> <select id="reconstitution_calculator_volume_unit" class="calculator_input calculator_select"> <option value="ml">ml</option> <option value="ul" selected="selected">ul</option> </select> </span> </div> <span class="calculator_equals calculator_operator">=</span> <div class="calc_section"> <label for="reconstitution_calculator_mass" class="calculator_label">Mass (in vial)</label> <input id="reconstitution_calculator_mass" name="reconstitution_calculator_mass" type="text" maxlength="16" placeholder="mass" class="calculator_input calculator_text"/> <span class="select_wrapper"> <select id="reconstitution_calculator_mass_unit" class="calculator_input calculator_select"> <option value="mg">mg</option> <option value="ug" selected="selected">ug</option> <option value="ng">ng</option> </select> </span> </div> <span class="calculator_divide calculator_operator">÷</span> <div class="calc_section"> <label for="reconstitution_calculator_concentration" class="calculator_label">Desired Reconstitution Concentration</label> <input id="reconstitution_calculator_concentration" name="reconstitution_calculator_concentration" type="text" maxlength="16" placeholder="conc." class="calculator_input calculator_text"/> <span class="select_wrapper"> <select id="reconstitution_calculator_concentration_unit" class="calculator_input calculator_select"> <option value="mg/ml">mg/ml</option> <option value="ug/ml" selected="selected">ug/ml</option> <option value="ng/ml">ng/ml</option> </select> </span> </div> <div id="reconstitution_foot" class="calc_section"> <input type="submit" value="Calculate" id="button_reconstitution_calculator_calculate" class="calculator_button btn btn-secondary"/> </div> </form> </div> </div></div> </div> <div id="ds_background" class="datasheet_section"> <h2>Background: Lysosomal alpha-Glucosidase</h2> <p>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).</p> <div class="ds_table background_table"> <div class="ds_table_body"> <div class="ds_table_row"> <div class="ds_table_cell background_table_name"><a id="reftop"></a>References</div> <div class="ds_table_cell background_table_data"><ol><li> Hoefsloot L.H. <em>et al</em>. (1988) EMBO J. <strong>7</strong>:1697. </li><li>Wan, L. <em>et al.</em> (2008) J. Neurol. <strong>255</strong>:831. </li><li>Fukuda, T. <em>et al</em>. (2007) Curr. Neurol. Neurosci. Rep. <strong>7</strong>:71. </li><li>Van Gelder, C.M. <em>et al.</em> (2014) J Inherit Metab Dis. In press. </li><li>Toscano, A. and Schoser, B. (2013) J. Neurol. <strong>260</strong>:951.</li></ol></div> </div> <div class="ds_table_row"> <div class="ds_table_cell background_table_name">Long Name</div> <div class="ds_table_cell background_table_data">Glucosidase, Alpha; Acid</div> </div> <div class="ds_table_row"> <div class="ds_table_cell background_table_name">Entrez Gene IDs</div> <div class="ds_table_cell background_table_data">2548 (Human); 14387 (Mouse); 367562 (Rat); 102141245 (Cynomolgus Monkey)</div> </div> <div class="ds_table_row"> <div class="ds_table_cell background_table_name">Alternate Names</div> <div class="ds_table_cell background_table_data">Acid alpha-Glucosidase; Acid Maltase; Aglucosidase alfa; EC 3.2.1.20; GAA; glucosidase, alpha; acid; LYAG; Lysosomal alphaGlucosidase; Lysosomal alpha-Glucosidase</div> </div> </div> </div> </div> <div id="ds_related_research_areas" class="datasheet_section"> <h2>Related Research Areas</h2> <ul class="ds_details"> <li> <a href="/research-area/glycobiology--related-enzymes">Glycobiology-related Enzymes</a> </li> <li> <a href="/research-area/lysosomal-enzymes">Lysosomal Enzymes</a> </li> <li> <a href="/research-area/search-bioprocessing-enzymes">Search Bioprocessing Enzymes</a> </li> <li> <a href="/research-area/search-glycobiology--related-enzymes">Search Glycobiology-related Enzymes</a> </li> </ul> </div> </div> <div class="tab_content tab_4" data-tab="4" data-tabname="product-faqs" id="product-faqs"> <h2>FAQs</h2> <div class="row"> <div class="col-xs-12"> <p>No product specific FAQs exist for this product, however you may</p> <a class="btn btn-quinary view_all_faqs_btn" href="/resources/faqs/proteins-and-enzymes"> View all Proteins and Enzyme FAQs </a> </div> </div> </div> <div class="tab_content tab_6" data-tab="6" data-tabname="product-reviews" id="reviews"> <h3>Reviews for Recombinant Human Lysosomal alpha-Glucosidase/GAA, CF</h3> <div class="row"> <div class="col-xs-12"> <div class="datasheet_section"> <div class="row"> <div class="col-md-6"> <p> Average Rating: 5 <span class="review_stars_5"></span> (Based on 4 Reviews) </p> <div class="review_rating_progress"> <div class="row"> <div class="col-xs-3"> 5 Star </div> <div class="col-xs-6"> <div class="progress"> <div class="progress-bar progress-bar-warning" role="progressbar" aria-valuenow="100" aria-valuemin="0" aria-valuemax="100" style="width:100%"> </div> </div> </div> <div class="col-xs-3"> 100% </div> </div> <div class="row"> <div class="col-xs-3"> 4 Star </div> <div class="col-xs-6"> <div class="progress"> <div class="progress-bar progress-bar-warning" role="progressbar" aria-valuenow="0" aria-valuemin="0" aria-valuemax="100" style="width:0%"> </div> </div> </div> <div class="col-xs-3"> 0% </div> </div> <div class="row"> <div class="col-xs-3"> 3 Star </div> <div class="col-xs-6"> <div class="progress"> <div class="progress-bar progress-bar-warning" role="progressbar" aria-valuenow="0" 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Yen for a review with an image</p> <p>$10/€7/£6/$10 CAD/¥70 Yuan/¥1110 Yen for a review without an image</p> </div> <a class="btn btn-quinary" href="/reviews/8329-GH"> Submit a Review </a> </div> </div> </div> </div> </div> </div> <div class="row"> <div class="col-md-12"> <div class="reviews_filters"> <p style="display:inline;"><b>Filter by:</b></p> <div class="select_wrapper"> <select class="review_filter_rating"> <option value="all">All Ratings</option> <option value="5">5</option> </select> </div> <div class="select_wrapper"> <select class="review_filter_application"> <option value="all">All Applications</option> <option value="Enzymatic activity in vitro"> Enzymatic activity in vitro </option> <option value="In vitro bioactivity in cell culture"> In vitro bioactivity in cell culture </option> </select> </div> </div> </div> </div> <br/> <div class="review_row" data-rating="5" data-species="" data-application="Enzymatic activity in vitro"> <div class="row"> <div class="col-xs-12 col-sm-10"> <div class="review_title review_stars_5">Recombinant Human Lysosomal alpha-Glucosidase/GAA, CF</div> <div class="review_date">By <span class="review_reviewer">Frank Martiniuk</span> on 05/02/2020 </div> <div class="row"> <div class="review_specifics col-xs-12"> <span class="ds_review_data"> <strong>Application:</strong> Enzymatic activity in vitro </span> <br/><span class="ds_review_data"> <strong>Reason for Rating: </strong> The rhGAA was very comparable to native placental GAA for activity, uptake by cell lines and various inhibitors. </span> </div> </div> </div> </div> <hr/> </div> <div class="review_row" data-rating="5" data-species="" data-application="In vitro bioactivity in cell culture"> <div class="row"> <div class="col-xs-12 col-sm-10"> <div class="review_title review_stars_5">Recombinant Human Lysosomal alpha-Glucosidase/GAA, CF</div> <div class="review_date">By <span class="review_reviewer">Anonymous</span> on 12/01/2017 </div> <div class="row"> <div class="review_specifics col-xs-12"> <span class="ds_review_data"> <strong>Application:</strong> In vitro bioactivity in cell culture </span> </div> </div> </div> </div> <hr/> </div> <div class="review_row" data-rating="5" data-species="" data-application="Enzymatic activity in vitro"> <div class="row"> <div class="col-xs-12 col-sm-10"> <div class="review_title review_stars_5">Recombinant Human Lysosomal alpha-Glucosidase/GAA, CF</div> <div class="review_date">By <span class="review_reviewer">Anonymous</span> on 05/23/2016 </div> <div class="row"> <div class="review_specifics col-xs-12"> <span class="ds_review_data"> <strong>Application:</strong> Enzymatic activity in vitro </span> </div> </div> </div> </div> <hr/> </div> <div class="review_row" data-rating="5" data-species="" data-application="Enzymatic activity in vitro"> <div class="row"> <div class="col-xs-12 col-sm-10"> <div class="review_title review_stars_5">Recombinant Human Lysosomal alpha-Glucosidase/GAA, CF</div> <div class="review_date">By <span class="review_reviewer">Anonymous</span> on 05/17/2016 </div> <div class="row"> <div class="review_specifics col-xs-12"> <span class="ds_review_data"> <strong>Application:</strong> Enzymatic activity in vitro </span> </div> </div> </div> </div> <hr/> </div> </div> </div> </div> <div id="sidebar" class="col-xs-12 col-sm-4 col-md-3 col-lg-3 col-xl-3"> <div class="distributor_region"></div> <div class="widget"> <div class="widget_title">Related Products</div> <div class="widget_content" id="related_products_area"> <ul class="ds_details"> <li> <a href="/target/lysosomal-alpha-glucosidase"> All Products for Lysosomal alpha-Glucosidase </a> </li> <li> <a href="/target/lysosomal-alpha-glucosidase?category=cDNA%20Clones"> Lysosomal alpha-Glucosidase cDNA Clones </a> </li> <li> <a href="/target/lysosomal-alpha-glucosidase?category=Primary%20Antibodies"> Lysosomal alpha-Glucosidase Primary Antibodies </a> </li> <li> <a 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