Results 181 to 190 of about 3,930 (214)

The Role of Glycosphingolipids in Autoimmune Manifestations and Myeloma in Gaucher Disease

Pavlova E, Archer J, Guimaraes Da Lomba Ferraz M, Aerts J, Cox T, the MRC Gaucherite consortium.   +5 more
europepmc   +1 more source

Current and Novel Aspects on the Non-lysosomal β-Glucosylceramidase GBA2

Neurochemical Research, 2015
The non-lysosomal β-glucosylceramidase GBA2 (EC3.2.1.45, GH116) is ubiquitously expressed in various mammal tissues and cell types where it catalyzes the hydrolysis of glucosylceramide into glucose and ceramide. Although it has been known for many years that the central nervous system is the main site of GBA2 expression and activity, little information
Massimo Aureli, Maura Samarani, Nicoletta Loberto, Giulia Mancini, Valentina Murdica, Elena Chiricozzi, Alessandro Prinetti, Rosaria Bassi, Sandro Sonnino   +8 more
semanticscholar   +6 more sources

Characterization of human glucosylsphingosine glucosyl hydrolase and comparison with glucosylceramidase

European Journal of Biochemistry, 1985
Properties of glucosylsphingosine (gluco‐psychosine) glucosyl hydrolase were studied in detail in cultured human fibroblasts and placenta and were compared with those of glucosylceramidase. The two activities, that are deficient in tissues of Gaucher patients, showed minor but consistent differences. The pH optima were 4.8 for psychosine hydrolysis and
Anna Maria Vaccaro, Michele Muscillo, Kunihiko Suzuki   +2 more
semanticscholar   +4 more sources

The Molecular Impact of Glucosylceramidase Beta 1 (Gba1) in Parkinson’s Disease: a New Genetic State of the Art

Molecular Neurobiology
Parkinson's disease (PD) is a neurodegenerative disorder affecting 2-3% of those aged over 65, characterized by motor symptoms like slow movement, tremors, and muscle rigidity, along with non-motor symptoms such as anxiety and dementia. Lewy bodies, clumps of misfolded proteins, contribute to neuron loss in PD. Mutations in the GBA1 gene are considered
Júlio César Claudino dos Santos, Gabriela Braga Cabrera Mano, André Rodrigues da Cunha Barreto Vianna, Túlia Fernanda Meira Garcia, Aline Vieira de Vasconcelos, Caio Sérgio Gomes Sá, Sarah Santana, Ana Gabriela Ponte Farias, Beatriz Seimaru, Micael Porto Portela Lima, João Vitor Caetano Goes, Camilla Teixeira Pinheiro Gusmão, Howard Lopes Ribeiro   +12 more
semanticscholar   +4 more sources

A nucleotide substitution in exon 8 of the glucosylceramidase beta gene is associated with Gaucher disease in sheep

Animal Genetics, 2017
Huitong Zhou, Yunsheng Zhang, Robert Suter, Hua Gong, Qian Fang, Ping Zhou, Jon G. H. Hickford   +6 more
semanticscholar   +4 more sources

Further studies on the reconstitution of glucosylceramidase activity by Sap C and anionic phospholipids [PDF]

FEBS Letters, 2000
The reconstitution of the activity of the lysosomal enzyme glucosylceramidase requires anionic phospholipids and, at least, a protein factor, saposin C (Sap C). We have previously proposed a mechanism for the glucosylceramidase activation [Vaccaro et al. (1993) FEBS Lett.
Rosa Salvioli, Massimo Tatti, Fiorella Ciaffoni, Anna Maria Vaccaro   +3 more
semanticscholar   +4 more sources

Accumulation of Tissue Glucosylsphingosine in Gaucher-like Mouse Induced by the Glucosylceramidase Inhibitor Cyclophellitol

Archives of Biochemistry and Biophysics, 1993
Shinichiro Atsumi, Chisato Nosaka, H. Iinuma, Kazuo Umezawa   +3 more
semanticscholar   +4 more sources

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Glucosylceramidase degradation in fibroblasts carrying bi-allelic Parkin mutations.

Molecular Genetics and Metabolism, 2013
A. McNeill, D. Healy, A. Schapira, J. Taanman   +3 more
semanticscholar   +3 more sources

Purification of glucosylceramidase by affinity chromatography.

Canadian Journal of Biochemistry, 1982
Glucosylceramide:β-glucosidase (glucocerebrosidase, EC 3.2.1.45) has been purified 12 900-fold from human placenta using a specific affinity column. The ligand, glucosyl sphingosine, prepared from glucocerebroside by alkaline hydrolysis, was attached to epoxy-activated Sepharose 6B.
P. Strasberg, J. Lowden, D. Mahuran
semanticscholar   +3 more sources