Results 61 to 70 of about 15,101 (190)

Visualization of Active Glucocerebrosidase in Rodent Brain with High Spatial Resolution following In Situ Labeling with Fluorescent Activity Based Probes.

PLoS ONE, 2015
Gaucher disease is characterized by lysosomal accumulation of glucosylceramide due to deficient activity of lysosomal glucocerebrosidase (GBA). In cells, glucosylceramide is also degraded outside lysosomes by the enzyme glucosylceramidase 2 (GBA2) of ...
Daniela Herrera Moro Chao, Wouter W Kallemeijn, Andre R A Marques, Marie Orre, Roelof Ottenhoff, Cindy van Roomen, Ewout Foppen, Maria C Renner, Martina Moeton, Marco van Eijk, Rolf G Boot, Willem Kamphuis, Elly M Hol, Jan Aten, Hermen S Overkleeft, Andries Kalsbeek, Johannes M F G Aerts   +16 more
doaj   +1 more source

Ambroxol Hydrochloride Improves Motor Functions and Extends Survival in a Mouse Model of Familial Amyotrophic Lateral Sclerosis

Frontiers in Pharmacology, 2019
Amyotrophic lateral sclerosis (ALS) is a multifactorial and fatal neurodegenerative disease. Growing evidence connects sphingolipid metabolism to the pathophysiology of ALS.
Alexandra Bouscary, Alexandra Bouscary, Cyril Quessada, Cyril Quessada, Althéa Mosbach, Althéa Mosbach, Noëlle Callizot, Michael Spedding, Jean-Philippe Loeffler, Jean-Philippe Loeffler, Alexandre Henriques, Alexandre Henriques, Alexandre Henriques   +12 more
doaj   +1 more source

Alterations in the properties of the cell membrane due to glycosphingolipid accumulation in a model of Gaucher disease [PDF]

, 2018
K
Batta, Gyula, Bozó, Tamás, Kellermayer, Miklós, Kovács, Tamás, Mészár, Zoltán Mihály, Nagy, Péter, Soltész, Lilla, Szöllősi, János   +7 more
core   +2 more sources

Indication for Light Sneutrinos and Gauginos from Precision Electroweak Data [PDF]

, 2001
The present Standard Model fit of precision data has a low confidence level, and is characterized by a few inconsistencies. We look for supersymmetric effects that could improve the agreement among the electroweak precision measurements and with the ...
Altarelli, G., Caravaglios, F., Gambino, P., Giudice, G. F., Ridolfi, G.   +4 more
core   +2 more sources

Delineating pathological pathways in a chemically-induced mouse model of Gaucher disease [PDF]

, 2016
Great interest has been shown in understanding the pathology of Gaucher disease (GD), due to the recently discovered genetic relationship with Parkinson's disease. For such studies, suitable animal models of GD are required.
Eilam, R, Futerman, AH, Kenwood, BM, Klein, AD, Massaro, G, Merrill, AH, Meshcheriakova, A, Rahim, AA, Vardi, A, Vitner, EB, Yaacobi, C, Zigdon, H   +11 more
core   +1 more source

Repression of autophagy leads to acrosome biogenesis disruption caused by a sub-chronic oral administration of polystyrene nanoparticles

Environment International, 2022
As a new widespread contaminant, nanoplastics (NPs) pose a potential risk to human health. Nevertheless, the adverse effects of NPs on the male reproductive system are poorly understood.
Lixiao Zhou, Ziying Yu, Yinyin Xia, Shuqun Cheng, Jieying Gao, Wei Sun, Xuejun Jiang, Jun Zhang, Lejiao Mao, Xia Qin, Zhen Zou, Jingfu Qiu, Chengzhi Chen   +12 more
doaj   +1 more source

Proteomic analysis of the zone of degeneration at the mitosis–meiosis transition stage in wild‐caught male catshark (Scyliorhinus canicula), correlated with an unusually high‐water temperature in the English Channel

Journal of Fish Biology, EarlyView.
Abstract In the context of current global change, variations in water temperature are one of the environmental conditions with serious consequences for marine life, including reproductive processes. In the small spotted catshark Scyliorhinus canicula, spermatogenesis occurs in spermatocysts composed of synchronously developing germ cells associated ...
Fabian Jeanne, Stanislas Pilet, Sean Heighton, Benoît Bernay, Pascal Sourdaine   +4 more
wiley   +1 more source

Revealing the roles of glycosphingolipid metabolism pathway in the development of keloid: a conjoint analysis of single-cell and machine learning

Frontiers in Immunology, 2023
Keloid is a pathological scar formed by abnormal wound healing, characterized by the persistence of local inflammation and excessive collagen deposition, where the intensity of inflammation is positively correlated with the size of the scar formation ...
Binyu Song, Yu Zheng, Hao Chi, Yuhan Zhu, Zhiwei Cui, Lin Chen, Guo Chen, Botao Gao, Yichen Du, Zhou Yu, Baoqiang Song   +10 more
doaj   +1 more source

The many origins of charge inversion in electrolyte solutions: effects of discrete interfacial charges

, 2007
We show that charge inversion, i.e. interfacial charges attracting counterions in excess of their own nominal charge, is a general effect that takes place in most charged systems next to aqueous solutions with multivalent ions and identify three ...
Faraudo, J., Travesset, A.
core   +1 more source

Cerebellar ataxia and sensory ganglionopathy associated with light-chain myeloma. [PDF]

, 2017
BACKGROUND: Cerebellar ataxia with sensory ganglionopathy is a rare neurological combination that can occur in some hereditary ataxias including mitochondrial diseases and in gluten sensitivity. Individually each condition can be a classic paraneoplastic
A Kumar, A Ramirez-Zamora, A Sghirlanzoni, A Wavre, C Denier, D Hilleman, F Graus, F Scaravilli, J Dalmau, J Dalmau, KG Gwathmey, M Hadjivassiliou, M Hadjivassiliou, M Pavan, P Zis, PD Shanmugarajah, PH Green, S Currie, T Alam   +18 more
core   +2 more sources