Linked Life Data

17510371

Source:http://linkedlifedata.com/resource/pubmed/id/17510371

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
10
pubmed:dateCreated
2007-9-18
pubmed:abstractText
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. Although the preservation by this method is excellent, the size of the tissue is limited to 1 mm(3). 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 (8 x 15 mm) while also 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 postfixation resulted in improved tissue morphology and glycogen preservation. Both techniques provide accurate anatomic evaluation of the glycogen distribution in Pompe mouse brain.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
0022-1554
pubmed:author
pubmed:issnType
Print
pubmed:volume
55
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
991-8
pubmed:meshHeading
pubmed:year
2007
pubmed:articleTitle
Optimized preservation of CNS morphology for the identification of glycogen in the Pompe mouse model.
pubmed:affiliation
Department of Pathology, Genzyme Corporation, One Mountain Road, Framingham, MA 01701-9322, USA. tatyana.taksir@genzyme.com
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't