Results 81 to 90 of about 36,633 (265)

Gaucher disease mouse models: point mutations at the acid β-glucosidase locus combined with low-level prosaposin expression lead to disease variants

Journal of Lipid Research, 2005
Gaucher disease is a common lysosomal storage disease caused by a defect of acid β-glucosidase (GCase). The optimal in vitro hydrolase activity of GCase requires saposin C, an activator protein that derives from a precursor, prosaposin.
Ying Sun, Brian Quinn, David P. Witte, Gregory A. Grabowski   +3 more
doaj   +1 more source

Elevation of Stearoyl‐Coenzyme A Desaturase and Monounsaturated Fatty Acids in Parkinson's Disease Serum

Movement Disorders, EarlyView.
Abstract Background Emerging evidence indicates that dysregulation of monounsaturated fatty acids (MUFAs), synthesized by the enzyme stearoyl‐coenzyme A desaturase (SCD), impacts on α‐synuclein pathology in the Parkinson's disease (PD) brain. Objective The objective of this study was to analyze SCD and MUFA‐enriched lipids in the periphery of patients ...
Finula I. Isik, Russell Pickford, Michelle Chua, Simon J.G. Lewis, Nicolas Dzamko, Woojin Scott Kim   +5 more
wiley   +1 more source

Glycosphingolipids from cultured astroblasts

Journal of Lipid Research, 1977
The glycolipids of two clonal lines astroblasts, NN clone and C6 clone, were studied. Glucosylceramide and lactosylceramide were present at very low levels in both clones, but the most common myelin glycolipids galactosylceramide and sulfatide, were not detected.
J Robert, G Rebel, P Mandel
openaire   +3 more sources

Characterization of moose intestinal glycosphingolipids [PDF]

Glycoconjugate Journal, 2015
As a part of a systematic investigation of the species-specific expression of glycosphingolipids, acid and non-acid glycosphingolipids were isolated from three small intestines and one large intestine of the moose (Alces alces). The glycosphingolipids were characterized by binding of monoclonal antibodies, lectins and bacteria in chromatogram binding ...
Johansson, Miralda Madar, Dedic, Benjamin, Lundholm, Klara, Branzell, Filip Berner, Barone, Angela, Benktander, John, Teneberg, Susann   +6 more
openaire   +2 more sources

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

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   +1 more source

GM1 Ganglioside Modifies α-Synuclein Toxicity and is Neuroprotective in a Rat α-Synuclein Model of Parkinson\u27s Disease. [PDF]

, 2019
While GM1 may interact with α-synuclein in vitro to inhibit aggregation, the ability of GM1 to protect against α-synuclein toxicity in vivo has not been investigated.
Aras, Radha, Brotchie, Jonathan M., Koprich, James B., Schneider, Jay S., Singh, Vikrant, Williams, Courtney K.   +5 more
core   +1 more source

Editorial: Membrane lipids in T cell functions [PDF]

, 2018
Plasma membrane lipids play essential roles in regulating T cell signaling, differentiation, and effector functions. The major lipid species in the plasma membrane are glycerophospholipids, sphingolipids, and sterol lipids.
Tuosto, Loretta, Xu, Chenqi
core   +1 more source

Heart failure in two male patients with late‐onset Fabry mutation (IVS4 + 919G > A)

ESC Heart Failure, Volume 12, Issue 2, Page 1508-1513, April 2025.
Xufei Yang, Chunlan Deng, Xiaogang Guo, Hui Yan   +3 more
wiley   +1 more source

Enhancing Lipidomics With High‐Resolution Ion Mobility‐Mass Spectrometry

PROTEOMICS, EarlyView.
ABSTRACT Lipids, indispensable yet structurally intricate biomolecules, serve as critical regulators of cellular function and disease progression. Conventional lipidomics, constrained by limited resolution for isomeric and low‐abundance species, has been transformed by ion mobility‐mass spectrometry (IM‐MS).
Gaoyuan Lu, Shuling Xu, Penghsuan Huang, Lingjun Li   +3 more
wiley   +1 more source

Atrial cardiomyopathy

ESC Heart Failure, Volume 12, Issue 2, Page 727-729, April 2025.
Wojciech Kosmala, Monika Przewłocka‐Kosmala   +1 more
wiley   +1 more source