نتایج جستجو برای: glucocerebrosidase

تعداد نتایج: 825  

2016
Matthew E. Gegg Anthony H.V. Schapira

The lysosomal hydrolase glucocerebrosidase (GCase) is encoded for by the GBA gene. Homozygous GBA mutations cause Gaucher disease (GD), a lysosomal storage disorder. Furthermore, homozygous and heterozygous GBA mutations are numerically the greatest genetic risk factor for developing Parkinson's disease (PD), the second most common neurodegenerative disorder. The loss of GCase activity results ...

2013
Neuza Teixeira Sriram Varahan Matthew J. Gorman Kelli L. Palmer Anna Zaidman-Remy Ryoji Yokohata Jiro Nakayama Lynn E. Hancock António Jacinto Michael S. Gilmore Maria de Fátima Silva Lopes

Enterococcus faecalis V583 is a vancomycin-resistant clinical isolate which belongs to the hospital-adapted clade, CC2. This strain harbours several factors that have been associated with virulence, including the fsr quorum-sensing regulatory system that is known to control the expression of GelE and SprE proteases. To discriminate between genes directly regulated by Fsr, and those indirectly r...

Journal: :Neonatology 2011
Sabine Haverkaemper Thorsten Marquardt Ingrid Hausser Katharina Timme Thomas Kuehn Christoph Hertzberg Rainer Rossi

This paper describes a neonate with type II Gaucher disease. The phenotype was unusually severe with congenital ichthyosis, hepatosplenomegaly, muscular hypotonia, myoclonus and respiratory failure. Electron microscopy of the skin revealed lamellar body contents in the stratum corneum interstices, appearances considered to be typical of type II Gaucher disease. The baby died from respiratory fa...

Journal: :Proceedings of the National Academy of Sciences 1985

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