Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease

Sandhoff disease is a severe neurodegenerative glycosphingolipid (GSL) lysosomal storage disorder, currently without treatment options.One therapeutic approach under investigation is substrate reduction therapy (SRT).By partially inhibiting GSL biosynthesis, the impaired rate of GSL catabolism is balanced by a slower rate of influx of GSLs into the

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Computational fluid dynamics approach for energy savings evaluation in core annular flow of a horizontal T-pipe

The application of core annular flow (CAF) has become an interesting solution in transporting heavy oil through pipeline because of its energy reduction and cost efficiency.At the same time, the CAF in pipe with hydraulic fittings and the problem of unstable CAF are the part of some big issues in using CAF techniques.This study intended NAPKIN to i

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Fate of Ukrainian fists: deportation, loss, attempts homecoming

The article refers to the Bolshevik genocide against prosperous Ukrainian peasantry, which the communist authorities, referring to the category of exploiters deported in the undeveloped eastern and northern regions of the USSR, to reduce resistance to forced collectivization of farmers.In particular archival documents highlighted the course of depo

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