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Analysis of galactocerebrosidase activity in the mouse brain by a new histological staining method
Analysis of galactocerebrosidase activity in the mouse brain by a new histological staining method JOURNAL OF NEUROSCIENCE RESEARCH Dolcetta, D., Perani, L., Givogri, M. I., Galbiati, F., Orlacchio, A., Martino, S., Roncarolo, M. G., Bongarzone, E. 2004; 77 (3): 462-464Abstract
Gene therapy of galactocerebrosidase (GALC) deficient mice (Twitcher mutants) requires a fast and sensitive assay to detect transduced cells in vitro and in vivo. We have developed a new rapid histochemical method that specifically detects GALC activity in situ in neural cells using 5-Br-3Cl-beta-galactopiranoside (X-Gal) in the presence of taurodeoxycholic and oleic acids to enhance suspension of the substrate at low pH. Using this method, we observed robust X-Gal staining in diverse neuronal populations and interfascicular oligodendrocytes in sections from normal mouse brain. In contrast, sections of Twitcher brain did not show a specific staining pattern in neurons or glial cells. The availability of this new sensitive and rapid in situ detection assay is fundamental for the follow-up of Twitcher mice under gene or cellular therapies to correct central GALC deficiency.
View details for DOI 10.1002/jnr.20169
View details for Web of Science ID 000222881300015
View details for PubMedID 15248301