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In Rodents</a></li> <li><a href="https://www.compbio.com/subcutaneous-implants-video/">Subcutaneous Implants + Video</a></li> </ul> </li> </ul> </div> </div> </div> <div id="primary" class="content-area medium-9 columns"> <div class="header-title"> <h1 class="entry-title">Pompe Disease</h1> </div> <main id="main" class="site-main" role="main"> <article id="post-4415" class="post-4415 page type-page status-publish hentry"> <div id="breadcrumb"> <div id="crumbs"><span class="crumb"><a href="https://www.compbio.com">Home</a></span> <i class="fa fa-angle-right"></i> <span class="current">Pompe Disease</span></div> </div> <div class="entry-content"> <div class="the-content-contain"> <div class="row builder-row content-row"> <div class="medium-12 columns"> <p> <img class="alignleft size-medium wp-image-4428" src="https://www.compbio.com/images/Pompe-Disease-Web-Site-Image-300x200.jpg?v=1713461572" alt="" width="300" height="200" srcset="https://www.compbio.com/images/Pompe-Disease-Web-Site-Image-300x200.jpg?v=1713461572 300w, https://www.compbio.com/images/Pompe-Disease-Web-Site-Image.jpg?v=1713461565 400w" sizes="(max-width: 300px) 100vw, 300px"> </p> <p> </p> <p> </p> <p> </p> <p> </p> <p> </p> <p class="p1"><strong>Pompe Disease</strong></p> <p class="p1">Pompe’s Disease or Glycogen Storage Disease II, is a rare, inherited fatal disease affecting infants. It is an autosomal recessive metabolic disorder which damages muscle and nerve cells throughout the body due to an accumulation of glycogen in the lysosome resulting from a deficiency of the lysosomal acid alpha-glucosidase enzyme. It is the only glycogen storage disease with a defect in lysosomal metabolism, and the first glycogen storage disease to be identified, in 1932 by the Dutch pathologist J. C. Pompe. There is no true treatment, but research is underway for Pompe’s and other similar diseases. Myozyme and Lumizyme are two approved treatments for Pompe’s (Sanofi-Genzyme) that are somewhat effective, but not curative. There are no relevant animal models.</p> <p><strong>Histopathology of Pompe’s Disease at CBI</strong></p> <p>CBI has experience preparing and evaluating the pathology from patients with Pompe’s Disease. We have recently developed unique histopathologic and ultrastructural methods to evaluate clinical biopsy samples from patients on clinical trials. We offer the following:</p> <p><strong>Histology Assessments</strong></p> <ul> <li>Paraffin, plastic and frozen sections of surgical biopsies from treated and untreated patients</li> <li>Special histochemical staining</li> <li>Proprietary novel method for PAS staining on Epon thin sections to identify intracellular glycogen</li> <li>Immunohistochemistry</li> <li>Histomorphometric assessment of intracytoplasmic vacuoles</li> </ul> <p><strong>Electron Microscopy</strong></p> <ul> <li>Ultrastructural sections with standard evaluation of photomicrographs</li> <li>Histomorphometric assessment of intracytoplasmic vacuoles, lysomes and glycogen accumulation</li> </ul> <p><strong>Pathology Assessment</strong></p> <ul> <li>ACVP board certified veterinary pathology experienced in the evaluation of these lesions</li> <li>Experienced research pathologist skilled in ultrastructure and histomorphometry</li> <li>Evaluation of histologic and ultrastructural specimens</li> <li>Histomorphometry and statistical analysis</li> <li>Photomicroscopy</li> <li>Complete report preparation</li> </ul> <p class="p1"><span class="s1"><span style="color: #800000;"><strong>↓ </strong></span>Below are some representative </span><span class="s2">histology with</span><span class="s1"> thin sections and TEM of GSD specimens that we prepared and evaluated in our </span><span class="s3">laboratorie</span> <span class="s2">laboratory</span><span class="s1">. Digital image analysis and histomorphometry were conducted on the TEM samples below.</span> <span style="color: #800000;"><strong>↓</strong></span></p> <p class="p1"> <img loading="lazy" class="alignleft size-medium wp-image-4434" src="https://www.compbio.com/images/TEM-Web-Image-300x200.jpg?v=1713461722" alt="" width="300" height="200" srcset="https://www.compbio.com/images/TEM-Web-Image-300x200.jpg?v=1713461722 300w, https://www.compbio.com/images/TEM-Web-Image.jpg?v=1713461716 400w" sizes="(max-width: 300px) 100vw, 300px"> </p> <p> <img loading="lazy" class="alignleft size-medium wp-image-4436" src="https://www.compbio.com/images/PAS-Web-Image-300x200.jpg?v=1713461811" alt="" width="300" height="200" srcset="https://www.compbio.com/images/PAS-Web-Image-300x200.jpg?v=1713461811 300w, https://www.compbio.com/images/PAS-Web-Image.jpg?v=1713461805 400w" sizes="(max-width: 300px) 100vw, 300px"><img loading="lazy" class="alignleft size-medium wp-image-4440" src="https://www.compbio.com/images/PAS2-Web-Site-300x197.jpg?v=1713461799" alt="" width="300" height="197" srcset="https://www.compbio.com/images/PAS2-Web-Site-300x197.jpg?v=1713461799 300w, https://www.compbio.com/images/PAS2-Web-Site.jpg?v=1713461906 400w" sizes="(max-width: 300px) 100vw, 300px"> </p> <p> </p> <p> </p> <p> </p> <p> </p> <p> </p> <p class="p1"> <span class="s1"><i>Left: TEM micrograph showing </i></span><span class="s2"><i>ultrastructural</i></span><span class="s1"><i> changes in the lysosomes typical of </i></span><span class="s2"><i>Pompe’s Disease</i></span><span class="s1"><i>.  Middle: PAS on ultrathin plastic sections of a Pompe’s Disease patient.  Right: PAS on ultrathin plastic sections of a severely affected Pompe’s </i></span><span class="s2"><i>Disease</i></span><span class="s1"><i> patient.</i></span> </p> <p><span style="color: #008080;"><strong>♦</strong></span></p> <p class="p1">Be sure to check out the movie:</p> <p class="p2"><span style="color: #008080;"><strong><span class="s1">EXTRAORDINARY MEASURES</span></strong></span></p> <p class="p2">The film is about parents who form a biotechnology company to develop a drug to save the lives of their children, who have a life-threatening disease. The film is based on the true story of John and Aileen Crowley, whose children <span class="s1">have Pompe’s </span><span class="s2">Disease.</span></p> <p id="firstHeading" class="firstHeading" lang="en"><a href="https://en.wikipedia.org/wiki/Glycogen_storage_disease_type_II"><strong>Glycogen Storage Disease Type II</strong></a></p> <p><a href="https://pompediseasestudy.com/about-pompe-disease?gclid=CjwKCAiAyfvhBRBsEiwAe2t_i6q0efaeAXSSWgJbkk6Fp8IhQ-YqLiI5_sasKfZhzJkGGPo5OOE71RoCExAQAvD_BwE"><strong>Study Details: WHO MAY QUALIFY</strong></a></p> <p><img loading="lazy" class="alignleft size-medium wp-image-4451" src="https://www.compbio.com/images/extraordinary-measures-web-200x300.jpg?v=1713461957" alt="" width="200" height="300"></p> <p> </p> <p> </p> <p> </p> <p> </p> <p> </p> <p> </p> <p> </p> <p class="p1"><strong>Contact Comparative Biosciences, Inc. to discuss a scientific study program to facilitate the evaluation of Pompe Disease Studies and Services.</strong></p> </div> </div> </div> </div> </article> </main> </div> </div> <div class="call-out"> <h3 class="content-left"><span class="line-one">Have a question</span> <span class="line-two">or need a quote?</span></h3> <svg version="1.1" id="Layer_1" xmlns="http://www.w3.org/2000/svg" xmlns:xlink="http://www.w3.org/1999/xlink" x="0px" y="0px" viewBox="0 0 100 85" style="enable-background:new 0 0 100 85;" xml:space="preserve"> <style type="text/css"> .st0{fill:#FFFFFF;} </style> <g> <path class="st0" d="M64.3,0.5H16c-8.8,0-16,7.2-16,16V44c0,8.4,6.6,15.4,14.9,16l-3.7,17.4L31.6,60h8.8V43.9 c0-8.7,7.1-15.8,15.8-15.8h24.2V16.5C80.3,7.7,73.1,0.5,64.3,0.5z"/> <path class="st0" d="M89.1,33h-33c-6,0-10.9,4.9-10.9,10.9v18.8c0,6,4.9,10.9,10.9,10.9h22.4l13.9,11.8l-2.5-11.9 c5.7-0.4,10.2-5.1,10.2-10.9V44C100,38,95.1,33,89.1,33z M85.5,49.3c2.2,0,4,1.8,4,4.1c0,2.2-1.8,4.1-4,4.1s-4-1.8-4-4.1 C81.5,51.1,83.3,49.3,85.5,49.3z M59.6,57.4c-2.2,0-4-1.8-4-4.1c0-2.2,1.8-4.1,4-4.1c2.2,0,4,1.8,4,4.1 C63.7,55.6,61.9,57.4,59.6,57.4z M68.5,53.4c0-2.2,1.8-4.1,4-4.1s4,1.8,4,4.1c0,2.2-1.8,4.1-4,4.1S68.5,55.6,68.5,53.4z"/> </g> </svg> <h3 class="content-right"><span>We can help!</span> <a href="https://www.compbio.com/contact-us/"><span class="cta-btn">Contact us</span></a></h3> </div> </div> </div> <footer id="colophon" class="site-footer row"> <div class="site-info medium-12 columns"> Comparative Biosciences, Inc. · <strong>Phone: 888-994-4879</strong><br> Copyright 2024 Comparative Biosciences, Inc. 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