Title : Ubiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model.

Pub. Date : 2014

PMID : 25551612






5 Functional Relationships(s)
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1 Gaucher disease is a lysosomal storage disease caused by defective activity of acid beta-glucosidase (GCase), which leads to the accumulation of its major substrates, glucosylceramide (GlcCer) and glucosylsphingosine (GlcSph) in many cells. Glucosylceramides glucosidase, beta, acid Mus musculus
2 Gaucher disease is a lysosomal storage disease caused by defective activity of acid beta-glucosidase (GCase), which leads to the accumulation of its major substrates, glucosylceramide (GlcCer) and glucosylsphingosine (GlcSph) in many cells. Glucosylceramides glucosidase, beta, acid Mus musculus
3 Gaucher disease is a lysosomal storage disease caused by defective activity of acid beta-glucosidase (GCase), which leads to the accumulation of its major substrates, glucosylceramide (GlcCer) and glucosylsphingosine (GlcSph) in many cells. Glucosylceramides glucosidase, beta, acid Mus musculus
4 Gaucher disease is a lysosomal storage disease caused by defective activity of acid beta-glucosidase (GCase), which leads to the accumulation of its major substrates, glucosylceramide (GlcCer) and glucosylsphingosine (GlcSph) in many cells. Glucosylceramides glucosidase, beta, acid Mus musculus
5 GCStg/Gba1 mice showed 2-3 fold increases in tissue GCS activity as well as accelerated GlcCer accumulation and the appearance of lipid-laden CD68 positive macrophages in visceral organs. Glucosylceramides glucosidase, beta, acid Mus musculus