|1.||Platt, Frances M: 4 articles (05/2008 - 05/2004)|
|2.||Seyfried, Thomas N: 2 articles (05/2008 - 05/2004)|
|3.||Kasperzyk, Julie L: 2 articles (05/2008 - 05/2004)|
|4.||Dwek, Raymond A: 2 articles (04/2005 - 08/2004)|
|5.||Butters, Terry D: 2 articles (04/2005 - 08/2004)|
|6.||Nicolis, Elena: 1 article (11/2008)|
|7.||Borgatti, Monica: 1 article (11/2008)|
|8.||Rizzotti, Paolo: 1 article (11/2008)|
|9.||Bezzerri, Valentino: 1 article (11/2008)|
|10.||Cabrini, Giulio: 1 article (11/2008)|
|1.||Sandhoff Disease (Sandhoff's Disease)
08/01/2004 - "Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease."
05/01/2008 - "NB-DGJ treatment, administered from postnatal day 2 (p-2) to p-5 (600 mg/kg/day)), significantly reduced total brain ganglioside and GM2 content in the Sandhoff disease (Hexb(-/-)) mice, but did not reduce the content of GA2. "
05/01/2008 - "N-butyldeoxygalactonojirimycin reduces brain ganglioside and GM2 content in neonatal Sandhoff disease mice."
08/01/2004 - "In the present study, a more selective compound, the galactose analogue N-butyldeoxygalactonojirimycin (NB-DGJ), was evaluated in the Sandhoff disease mouse model. "
|2.||Gaucher Disease (Gaucher's Disease)
10/28/1994 - "In an in vitro Gaucher's disease model NB-DGJ was as effective as NB-DNJ in preventing glycolipid storage and may represent a more selective potential therapeutic agent than NB-DNJ for the management of this and other glycosphingolipidoses."
11/01/2008 - "Since both evidence support a link between CFTR function and inflammation, we extended our investigation to other F508del-CFTR correctors, such as miglustat (Norez, 2006), an approved drug for Gaucher disease, in comparison with the galactose analogue NB-DGJ. "
|3.||GM1 Gangliosidosis (Gangliosidosis GM1)
05/01/2004 - "N-butyldeoxygalactonojirimycin reduces neonatal brain ganglioside content in a mouse model of GM1 gangliosidosis."
05/01/2004 - "These findings suggest that substrate reduction therapy using NB-DGJ may be an effective early intervention for GM1 gangliosidosis and possibly other GSL lysosomal storage diseases."
|4.||Lysosomal Storage Diseases (Lysosomal Storage Disease)
|5.||Weight Loss (Weight Reduction)