|  Help  |  About  |  Contact Us

Publication : Optimization of a histopathological biomarker for sphingomyelin accumulation in acid sphingomyelinase deficiency.

First Author  Taksir TV Year  2012
Journal  J Histochem Cytochem Volume  60
Issue  8 Pages  620-9
PubMed ID  22614361 Mgi Jnum  J:214285
Mgi Id  MGI:5588657 Doi  10.1369/0022155412451129
Citation  Taksir TV, et al. (2012) Optimization of a histopathological biomarker for sphingomyelin accumulation in acid sphingomyelinase deficiency. J Histochem Cytochem 60(8):620-9
abstractText  Niemann-Pick disease (types A and B), or acid sphingomyelinase deficiency, is an inherited deficiency of acid sphingomyelinase, resulting in intralysosomal accumulation of sphingomyelin in cells throughout the body, particularly within those of the reticuloendothelial system. These cellular changes result in hepatosplenomegaly and pulmonary infiltrates in humans. A knockout mouse model mimics many elements of human ASMD and is useful for studying disease histopathology. However, traditional formalin-fixation and paraffin embedding of ASMD tissues dissolves sphingomyelin, resulting in tissues with a foamy cell appearance, making quantitative analysis of the substrate difficult. To optimize substrate fixation and staining, a modified osmium tetroxide and potassium dichromate postfixation method was developed to preserve sphingomyelin in epon-araldite embedded tissue and pulmonary cytology specimens. After processing, semi-thin sections were incubated with tannic acid solution followed by staining with toluidine blue/borax. This modified method provides excellent preservation and staining contrast of sphingomyelin with other cell structures. The resulting high-resolution light microscopy sections permit digital quantification of sphingomyelin in light microscopic fields. A lysenin affinity stain for sphingomyelin was also developed for use on these semi-thin epon sections. Finally, ultrathin serial sections can be cut from these same tissue blocks and stained for ultrastructural examination by electron microscopy.
Quick Links:
 
Quick Links:
 

Expression

Publication --> Expression annotations

 

Other

3 Bio Entities

Trail: Publication

0 Expression