Results 221 to 230 of about 7,739 (261)

Effects of switching from agalsidase-α to agalsidase-β on biomarkers, renal and cardiac parameters, and disease severity in fabry disease forming neutralizing antidrug antibodies: a case report. [PDF]

CEN Case Rep
Shima H, Tsukimura T, Shiga T, Togawa T, Sakuraba H, Doi T, Ikeda Y, Okamoto T, Yoshikawa Y, Kimura T, Iwase T, Inoue T, Tashiro M, Okada K, Minakuchi J.   +14 more
europepmc   +1 more source

Bilateral avascular necrosis: A rare complication of Fabry disease. [PDF]

Mol Genet Metab Rep
Romano C, Wells J, Stanzione N, Kimonis V.   +3 more
europepmc   +1 more source

Simultaneous Determination of Lyso-Gb1 and Lyso-Gb3 in Plasma Using Salt-Assisted Liquid-Liquid Extraction Combined with LC-MS/MS. [PDF]

ACS Omega
Canbay E, Uzun Ç, Akçay Y, Canda E, Yazıcı H, Kalkan Uçar S, Çoker M, Yıldırım Sözmen E.   +7 more
europepmc   +1 more source

Treatment with Agalsidase Alfa during Pregnancy in a Heterozygous Female with Fabry Disease

, 2016
BIFULCO, GIUSEPPE, DI SPIEZIO SARDO, ATTILIO, PISANI, ANTONIO, RICCIO, ELEONORA   +3 more
core  

Terapia enzimatica sostitutiva nella malattia di Fabry: cosa abbiamo imparato dallo switch?

, 2014
Pisani, Antonio, Riccio, Eleonora
core  

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Efficient Delivery of Globotriaosylceramide Synthase siRNA using Polyhistidine‐Incorporated Lipid Nanoparticles

Macromolecular Bioscience, 2023
AbstractIn this study, a novel polyhistidine‐incorporated lipid nanoparticle (pHis/LNP) is developed for the delivery of therapeutic globotriaosylceramide (Gb3) synthase siRNAs using a microfluidic device with pHis as a biocompatible method of endosome escape.
In Gyu Kim, Won Ho Jung, Gayeon You, Hyukjin Lee, Yoo Jin Shin, Sun Woo Lim, Byung Ha Chung, Hyejung Mok   +7 more
openaire   +3 more sources

Globotriaosylceramide inhibits iNKT‐cell activation in a CD1d‐dependent manner

European Journal of Immunology, 2015
Globotriaosylceramide (Gb3) is a glycosphingolipid present in cellular membranes that progressively accumulates in Fabry disease. Invariant Natural Killer T (iNKT) cells are a population of lipid‐specific T cells that are phenotypically and functionally altered in Fabry disease.
Pereira, C. S., Sa-Miranda, C., De Libero, G., Mori, L., Macedo, M. F.   +4 more
openaire   +4 more sources

The lipid globotriaosylceramide promotes germinal center B cell responses and antiviral immunity

Science
Influenza viruses escape immunity owing to rapid antigenic evolution, which requires vaccination strategies that allow for broadly protective antibody responses. We found that the lipid globotriaosylceramide (Gb3) expressed on germinal center (GC) B cells is essential for the production of high-affinity antibodies.
Pankaj Sharma, Xiaolong Zhang, Kevin Ly, Yuxiang Zhang, Yu Hu, Adam Yongxin Ye, Jianqiao Hu, Ji Hyung Kim, Mumeng Lou, Chong Wang, Quinton Celuzza, Yuji Kondo, Keiko Furukawa, David R. Bundle, Koichi Furukawa, Frederick W. Alt, Florian Winau   +16 more
openaire   +3 more sources

Lyso-globotriaosylceramide downregulates KCa3.1 channel expression to inhibit collagen synthesis in fibroblasts

Biochemical and Biophysical Research Communications, 2015
Fabry disease is an X-linked lysosomal storage disorder that is caused by a deficiency of α-galactosidase A. The disease ultimately manifests as multiple organ dysfunctions owing to excessive accumulation of globotriaosylceramide (Gb3). Among the several complications of Fabry disease, ascending thoracic aortic aneurysm is relatively common, which is ...
Ju Yeon, Choi, Mee-Young, Shin, Suk Hyo, Suh, Seonghee, Park   +3 more
openaire   +3 more sources

Increased globotriaosylceramide in familial dysautonomia

Lipids, 1992
AbstractFamilial Dysautonomia (FD) is an autosomal recessive disease of unknown etiology, occurring primarily in Ashkenazi Jews. Patients are neurologically impaired, with deficits primarily in autonomic and sensory functions. The biochemical and genetic defects have remained elusive, precluding carrier detection and prenatal diagnosis.
P, Strasberg, H, Yeger, I, Warren
openaire   +2 more sources