Results 1 to 10 of about 7,739 (261)

Role of Globotriaosylceramide in Physiology and Pathology [PDF]

Frontiers in Molecular Biosciences, 2022
At first glance, the biological function of globoside (Gb) clusters appears to be that of glycosphingolipid (GSL) receptors for bacterial toxins that mediate host-pathogen interaction.
Ana Beatriz Celi, Ana Beatriz Celi, Jorge Goldstein, Jorge Goldstein, María Victoria Rosato-Siri, Alipio Pinto, Alipio Pinto   +6 more
doaj   +6 more sources

Globotriaosylceramide Gb3 Influences Wound Healing and Scar Formation by Orchestrating Fibroblast Heterogeneity [PDF]

Advanced Science
Cutaneous fibroblast heterogeneity is mechanistically linked to wound repair outcomes and fibrotic progression, with glycosphingolipid metabolism emerging as a critical determinant of physiological fibroblast diversity.
Sujie Xie, Runzhi Huang, Weijin Qian, Xinran Ding, Wei Zhang, Yixu Li, Jianyu Lu, Hanlin Sun, Yifan Liu, Yuntao Yao, Bingnan Lu, Minjuan Wu, Zhaofan Xia, Shizhao Ji   +13 more
doaj   +4 more sources

Ceria-Zirconia nanoparticles reduce intracellular globotriaosylceramide accumulation and attenuate kidney injury by enhancing the autophagy flux in cellular and animal models of Fabry disease [PDF]

Journal of Nanobiotechnology, 2022
Background Fabry disease (FD) is a lysosome storage disease (LSD) characterized by significantly reduced intracellular autophagy function. This contributes to the progression of intracellular pathologic signaling and can lead to organ injury ...
Jong Hun An, Sang-Eun Hong, Seong-Lan Yu, Jaeku Kang, Chang Gyo Park, Hoi Young Lee, Sung-Ki Lee, Dong Chul Lee, Hwan-Woo Park, Won-Min Hwang, Sung-Ro Yun, Yohan Park, Moon Hyang Park, Kuk Ro Yoon, Se-Hee Yoon   +14 more
doaj   +3 more sources

In vivo demonstration of globotriaosylceramide brain accumulation in Fabry Disease using MR Relaxometry. [PDF]

Neuroradiology
Abstract Purpose How to measure brain globotriaosylceramide (Gb3) accumulation in Fabry Disease (FD) patients in-vivo is still an open challenge. The objective of this study is to provide a quantitative, non-invasive demonstration of this phenomenon using quantitative MRI (qMRI). Methods
Pontillo G, Tranfa M, Scaravilli A, Monti S, Capuano I, Riccio E, Rizzo M, Brunetti A, Palma G, Pisani A, Cocozza S.   +10 more
europepmc   +6 more sources

Sex differences of urinary and kidney globotriaosylceramide and lyso-globotriaosylceramide in Fabry mice [PDF]

Journal of Lipid Research, 2011
The aim of our study was to measure globotriaosylceramide (Gb3) and lyso-Gb3 levels by tandem mass spectrometry in the urine and kidney in Fabry (gla knockout) mice and wild-type controls.
Brandon Durant, Sabrina Forni, Lawrence Sweetman, Nastry Brignol, Xing-Li Meng, Elfrida R. Benjamin, Raphael Schiffmann, Jin-Song Shen   +7 more
doaj   +3 more sources

Accumulation of globotriaosylceramide in a case of leiomyosarcoma [PDF]

Biochemical Journal, 1986
Analysis of the glycosphingolipid composition in one case of uterine leiomyosarcoma metastasized to the liver showed an accumulation of globotriaosylceramide as compared with normal liver and uterus from which the tumour originated. The structure and the amount of glycosphingolipids were established by using specific glycosidases, permethylation ...
S C Li, S K Kundu, R DeGasperi, Y T Li
openalex   +4 more sources

Carbon-13 nuclear magnetic resonance spectrometry of globotriaosylceramide

Journal of Lipid Research, 1982
Resonances in the carbon-13 natural abundance, proton-decoupled, 90.5 MHz nuclear magnetic resonance spectrum of globotriaosylceramide were assigned to specific carbon nuclei. The chemical shifts were rationalized in terms of the number of sugar residues,
H A Nunez, C C Sweeley
doaj   +3 more sources

The glycosphingolipid globotriaosylceramide in the metastatic transformation of colon cancer [PDF]

Proceedings of the National Academy of Sciences, 2005
The most devastating aspect of cancer is the emergence of metastases. Thus, identification of potentially metastatic cells among a tumor cell population and the underlying molecular changes that switch cells to a metastatic state are among the most important issues in cancer biology.
Olga Kovbasnjuk, Rakhilya Mourtazina, Boris Baibakov, Thomas J. Wang, Christian Elowsky, Michael A. Choti, Anne Kane, Mark Donowitz   +7 more
openalex   +3 more sources

Reduction of globotriaosylceramide in Fabry disease mice by substrate deprivation [PDF]

Journal of Clinical Investigation, 2000
We used a potent inhibitor of glucosylceramide synthase to test whether substrate deprivation could lower globotriaosylceramide levels in alpha-galactosidase A (alpha-gal A) knockout mice, a model of Fabry disease. C57BL/6 mice treated twice daily for 3 days with D-threo-1-ethylendioxyphenyl-2-palmitoylamino-3-pyrrolidi no-propanol (D-t-EtDO-P4) showed
Akira Abe, Susan A. Gregory, Lihsueh Lee, Paul D. Killen, Roscoe O. Brady, Ashok B. Kulkarni, James A. Shayman   +6 more
openalex   +3 more sources

The importance of a multidisciplinary approach in two tricky cases: the perfect match for Fabry disease [PDF]

BMC Nephrology
Anderson-Fabry disease (AFD) is a multisystem X-linked lysosomal storage disorder caused by a deficiency in the enzyme α-galactosidase A (α-Gal A). This deficiency results in the intracellular accumulation of glycosphingolipids, primarily uncleaved ...
Gian Marco Berti, Valeria Aiello, Gisella Vischini, Sarah Lerario, Francesca Ciurli, Marisa Santostefano, Vincenzo Donadio, Elena Biagini, Michela Fresina, Benedetta Fabbrizio, Francesca Montanari, Daniela Turchetti, Gianandrea Pasquinelli, Renzo Mignani, Gaetano La Manna, Irene Capelli   +15 more
doaj   +2 more sources