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JHC exPRESS: First Published May 17, 2007. doi:10.1369/jhc.7A7239.2007
Copyright © Histochemical Society, Inc.

A more recent version of this article appeared on October 1, 2007.
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Optimized Preservation of CNS Morphology for the Identification of Glycogen in the Pompe Mouse Model

Tatyana V. Taksir 1*, Denise Griffiths 1, Jennifer Johnson 1, Susan Ryan 1, Lamya S. Shihabuddin 1 and Beth L. Thurberg 1

1 Departments of Pathology (TVT,DG,JJ,SR,BLT) and Neurobiology (LSS), Genzyme Corporation, Framingham, Massachusetts

* To whom correspondence should be addressed. E-mail: tatyana.taksir{at}genzyme.com.

Submitted on March 16, 2007
Accepted on 4 May 2007


  Abstract
Pompe disease (glycogenosis type II) is a rare lysosomal disorder caused by a mutational deficiency of acid {alpha}-glucosidase (GAA). This deficiency leads to glycogen accumulation in multiple tissues: heart, skeletal muscles, and the central nervous system. A knockout mouse model mimicking the human condition has been used for histological evaluation. Currently, the best method for preserving glycogen in Pompe samples uses epon-araldite resin. While the preservation by this method is excellent, the size of the tissue is limited to 1 cubic millimeter. To accurately evaluate brain pathology in the Pompe mouse model, a modified glycol methacrylate (JB-4 Plus) method was developed. This approach allowed the production of larger tissue sections encompassing an entire mouse hemisphere (8x15 mm) while providing a high level of morphological detail and preservation of glycogen. Application of the JB-4 Plus method is appropriate when a high level of cellular detail is desired. A modified paraffin method was also developed for use when rapid processing of multiple samples is a priority. Traditional paraffin processing results in glycogen loss. The modified paraffin method with periodic acid post-fixation resulted in improved tissue morphology and glycogen preservation. Both techniques provide accurate anatomic evaluation of the glycogen distribution in Pompe mouse brain.

Key Words: Pompe disease, glycogen preservation, paraffin, periodic acid, glycol methacrylate


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