Results 21 to 30 of about 7,739 (261)

In vitro effect of globotriaosylceramide on electron transport chain complexes and redox parameters

Anais da Academia Brasileira de Ciências, 2019
: Fabry disease (FD) is an X-linked inherited disease and occurs due to mutations in GLA gene that encodes the α-galactosidase enzyme. Consequently, there is an accumulation of enzyme substrates, namely globotriaosylceramide (GB3).
RAFAELA M. ALVARIZ, ISABEL T.D.S. MOREIRA, GABRIELA K. CURY, CARMEN R. VARGAS, ALETHÉA G. BARSCHAK   +4 more
doaj   +2 more sources

Globotriaosylceramide, Gb3, is an alternative functional receptor for Shiga-like toxin 2e [PDF]

Infection and Immunity, 1995
We reexamined the binding specificity of the Shiga-like toxin variant associated with porcine edema disease, SLT2e, which is reported to be more cytotoxic for Vero cells than for HeLa cells, by using receptor-deficient cells and a liposomal insertion system for purified glycolipids.
Gerald T. Keusch, Mary Jacewicz, David W. K. Acheson, Arthur Donohue‐Rolfe, Anne Kane, Robert H. McCluer   +5 more
openalex   +3 more sources

A novel unbiased method reveals progressive podocyte globotriaosylceramide accumulation and loss with age in females with Fabry disease

Kidney International, 2022
Behzad Najafian, Aurelio Silvestroni, Alexey Sokolovskiy, Camilla Tøndel, Einar Svarstad, Bogdan Obrișcă, Gener Ismail, Myrl Holida, Michael Mauer   +8 more
openalex   +3 more sources

The effects of globotriaosylceramide tail saturation level on bilayer phases

Soft Matter, 2015
The globotriaosylceramide acyl chains from one leaflet interdigitate into the opposing leaflet and lead to significant bilayer rigidification and immobilisation of the lipid tails. Globotriaosylceramide with saturated acyl chains can form a highly ordered, relatively immobile phase which is resistant to bending.
Weria Pezeshkian, Vitaly V. Chaban, Ludger Johannes, Julian Shillcock, John H. Ipsen, Himanshu Khandelia   +5 more
openaire   +5 more sources

Accumulation of Globotriaosylceramide in Podocytes in Fabry Nephropathy Is Associated with Progressive Podocyte Loss

Journal of the American Society of Nephrology, 2020
BACKGROUND In males with classic Fabry disease, the processes leading to the frequent outcome of ESKD are poorly understood. Defects in the gene encoding α-galactosidase A lead to accumulation of globotriaosylceramide (GL3) in various cell types.
Behzad Najafian, Camilla Tøndel, Einar Svarstad, Marie-Claire Gübler, João Paulo Oliveira, Michael Mauer   +5 more
openalex   +3 more sources

Risk of Death in Heart Disease is Associated With Elevated Urinary Globotriaosylceramide [PDF]

Journal of the American Heart Association, 2014
Background Elevated urinary globotriaosylceramide (Gb 3 ) has been considered a hallmark of F abry disease, an X‐linked lysosomal disorder that is a risk factor for most types of heart disease.
Schiffmann, R., Forni, S., Swift, C., Brignol, N., Wu, X., Lockhart, D., Blankenship, D., Wang, X., Grayburn, P., Taylor, M., Lowes, B., Fuller, M., Benjamin, E., Sweetman, L.   +13 more
openaire   +4 more sources

Fabry disease in W162C mutation: a case report of two patients and a review of literature [PDF]

BMC Neurology
Background Fabry disease is a multisystemic disorder characterized by deposition of globotriaosylceramide (Gb3) and its deacylated form in multiple organs, sometimes localized in specific systems such as the nervous or cardiovascular system.
Alessandro Furia, Raffaello Ditaranto, Elena Biagini, Vanda Parisi, Alex Incensi, Sara Parisini, Rocco Liguori, Vincenzo Donadio   +7 more
doaj   +2 more sources

Globotriaosylceramide-induced reduction of KCa1.1 channel activity and activation of the Notch1 signaling pathway in skin fibroblasts of male Fabry patients with pain

Experimental Neurology, 2019
Vanessa Rickert, Daniela Kramer, Anna‐Lena Schubert, Claudia Sommer, Erhard Wischmeyer, Nurcan Üçeyler   +5 more
openalex   +2 more sources

Globotriaosylceramide as a potential biomarker for auxiliary detection of lower respiratory tract infections of <i>Pseudomonas aeruginosa</i>. [PDF]

Exp Ther Med
Xu T, Li J, Dong Y, Zhao Z, Yu L.
europepmc   +3 more sources

Glucosylceramide synthase inhibition with lucerastat lowers globotriaosylceramide and lysosome staining in cultured fibroblasts from Fabry patients with different mutation types [PDF]

Human Molecular Genetics, 2018
Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the GLA gene coding for α-galactosidase A (α-GalA). The deleterious mutations lead to accumulation of α-GalA substrates, including globotriaosylceramide (Gb3) and ...
Richard W.D. Welford, Andreas Mühlemann, Marco Garzotti, Vanessa Rickert, Peter Groenen, O. Morand, Nurcan Üçeyler, Markus R. Probst   +7 more
openalex   +2 more sources