Results 71 to 80 of about 24,979 (220)

Pre-diagnosing and managing patients with GM1 gangliosidosis and related disorders by the evaluation of GM1 ganglioside content [PDF]

, 2019
GM1 ganglioside, a monosialic glycosphingolipid and a crucial component of plasma membranes, accumulates in lysosomal storage disorders, primarily in GM1 gangliosidosis.
Pavone, F. S., Ricci, Silvia, Morrone, Amelia, Caciotti, Anna, Tonin, Rodolfo, Mangone, Giusi, Ricci, S., Casini, Arianna, Ardissone, Anna, Caciotti, A., Procopio, Elena, Calamai, Martino, Marangi, Antonio, Di Rocco, Maja, Morrone, A., Salviati, A., Mancardi, Maria Margherita, Pavone, Francesco Saverio, Parini, Rossella, Guerrini, Renzo, Fischetto, Rita, Deodato, Federica, Salviati, Alessandro, Parini, R., Strisciuglio, Pietro, Fiumara, Agata, Tonin, R., Guerrini, R., Calamai, M.   +28 more
core   +1 more source

QUANTITATION OF GM1-β-GALACTOSIOASE (β-gal) CROSS REACTING MATERIAL (CRM) IN GM1-GANGLIOSIDOSES (GM1-gang) [PDF]

Pediatric Research, 1977
QUANTITATION OF GM 1 -β-GALACTOSIOASE (β-gal) CROSS REACTING MATERIAL (CRM) IN GM 1 -GANGLIOSIDOSES (GM 1 -gang)
Yoav Ben-Yoseph, Barbara K Burton, Henry L Nadlar   +2 more
openaire   +1 more source

Understanding extracellular vesicle biology, isolation, and characterization: The theory of EV‐erything

Periodontology 2000, EarlyView.
Abstract Background Extracellular vesicles (EVs), especially exosomes, are nanoparticles increasingly recognized as key regulators of intercellular communication in both physiological and pathological aspects. Despite rapid progress, inconsistencies in nomenclature, isolation, and characterization continue to restrict translational advancement.
Paras Ahmad, Nima Farshidfar, Nathan E. Estrin, Yulan Wang, Yufeng Zhang, Anton Sculean, Richard J. Miron   +6 more
wiley   +1 more source

GM1 improves neurofascin155 association with lipid rafts and prevents rat brain myelin injury after hypoxia-ischemia

Brazilian Journal of Medical and Biological Research, 2011
White matter injury characterized by damage to myelin is an important process in hypoxic-ischemic brain damage (HIBD). Because the oligodendrocyte-specific isoform of neurofascin, neurofascin 155 (NF155), and its association with lipid rafts are ...
Y.P. Zhang, Q.L. Huang, C.M. Zhao, J.L. Tang, Y.L. Wang   +4 more
doaj   +1 more source

Effects of cyclodextrins on GM1-gangliosides in fibroblasts from GM1-gangliosidosis patients

Journal of Pharmacy and Pharmacology, 2015
Abstract Objectives GM1-gangliosidosis is an inherited disorder characterized by the accumulation of GM1-gangliosides in many tissues and organs, particularly in the brain. Currently, there is no treatment available for patients with ganglioside storage diseases. Therefore, we investigated the effects
Yuki, Maeda, Keiichi, Motoyama, Taishi, Higashi, Yuka, Horikoshi, Toru, Takeo, Naomi, Nakagata, Yuki, Kurauchi, Hiroshi, Katsuki, Yoichi, Ishitsuka, Yuki, Kondo, Tetsumi, Irie, Hirokazu, Furuya, Takumi, Era, Hidetoshi, Arima   +13 more
openaire   +2 more sources

Image Encoding With Blipped Gradients in an Echo Train

Magnetic Resonance in Medicine, Volume 96, Issue 5, Page 2530-2538, November 2026.
ABSTRACT Purpose An MRI scanner was designed and built to encode k‐space points in the spin echoes occurring between 180∘ pulses in an echo train by using blipped B0 gradient pulses applied just prior to those spin echoes. Methods The proposed MRI scanner is a modification of an original design that used TRansmit Array Spatial Encoding (TRASE), a built‐
Logi Vidarsson, Gordon E. Sarty
wiley   +1 more source

Radiotherapy Enhances the Oncolytic Efficacy of the Novel Oncolytic Herpesvirus VG161 and Amplifies Its Antitumor Immunity in Breast Cancer

Advanced Science, Volume 13, Issue 52, 18 September 2026.
RT promotes VG161 replication in BC and its immunostimulatory transgenes expression, which is mediated by the upregulation of GADD34 and HVEM caused by RT. The combination therapy with VG161 and RT increases the abundances of tumor‐infiltrating lymphocytes and elicits potent systemic antitumor immunity, thereby effectively inhibiting local tumors and ...
Lijuan Lyu, Ming Yi, Ji Chen, Zeda Zhao, Ruocen Liao, Yingnan Wang, Shengtao Hu, Jun Ding, Ronghua Zhao, William Jia, Yinan Shen, Yongchang Wei, Kongming Wu, Zhijun Dai   +13 more
wiley   +1 more source

Generation of an infantile GM1 gangliosidosis induced pluripotent stem cell line (CHOCi005-A) for disease modeling and therapeutic testing

Stem Cell Research
GM1 gangliosidosis (GM1) is a rare autosomal recessive neurogenerative lysosomal storage disease characterized by deficiency of beta-galactosidase (β-gal) and intralysosomal accumulation of GM1 ganglioside and other glycoconjugates.
Allisandra K. Rha, Chloe L. Christensen, Shih-Hsin Kan, Jerry F. Harb, Perla Andrade-Heckman, Raymond Y. Wang   +5 more
doaj   +1 more source

Lipidomic Evaluation of Feline Neurologic Disease after AAV Gene Therapy

Molecular Therapy: Methods & Clinical Development, 2017
GM1 gangliosidosis is a fatal lysosomal disorder, for which there is no effective treatment. Adeno-associated virus (AAV) gene therapy in GM1 cats has resulted in a greater than 6-fold increase in lifespan, with many cats remaining alive at >5.7 years of
Heather L. Gray-Edwards, Xuntian Jiang, Ashley N. Randle, Amanda R. Taylor, Taylor L. Voss, Aime K. Johnson, Victoria J. McCurdy, Miguel Sena-Esteves, Daniel S. Ory, Douglas R. Martin   +9 more
doaj   +1 more source

Studies on Heterogeneity in GM1-Gangliosidosis [PDF]

, 1980
The activity of GM1-β-galactosidase in leukocytes and in lymphoblastoid cell lines transformed by Epstein-Barr virus, from five patients with reduced β-galactosidase activity and their parents, was assayed with GM1-ganglioside tritiated in the terminal ...
菊地, 浩一
core   +1 more source