Gaucher Disease—Correlation of Lyso-Gb1 with Haematology and Biochemical Parameters [PDF]
Background/Objectives: Gaucher disease (GD) is a lysosomal disorder caused by a deficiency of β-glucosidase. Disease-modifying therapies (DMTs) include enzyme replacement therapy (ERT) and substrate reduction therapy (SRT). Glucosylsphingosine (lyso-Gb1)
Simona D’Amore +3 more
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Increased Lyso-Gb1 Levels in an Obese Splenectomized Gaucher Disease Type 1 Patient Treated with Eliglustat: Unacknowledged Poor Compliance or Underlying Factors [PDF]
Eliglustat (Cerdelga®) is a potent and specific inhibitor of the enzyme glucosylceramide synthase and serves as a substrate reduction therapy for adult patients with Gaucher disease type 1 (GD1).
Evelina Maines +8 more
doaj +4 more sources
Lyso-GM2 ganglioside: a possible biomarker of Tay-Sachs disease and Sandhoff disease. [PDF]
To find a new biomarker of Tay-Sachs disease and Sandhoff disease. The lyso-GM2 ganglioside (lyso-GM2) levels in the brain and plasma in Sandhoff mice were measured by means of high performance liquid chromatography and the effect of a modified ...
Takashi Kodama +11 more
doaj +3 more sources
Preparation of deacetyl-, lyso-, and deacetyl-lyso-GM3 by selective alkaline hydrolysis of GM3 ganglioside [PDF]
Three methods (using GM3 quantities ranging from a few milligrams to grams) have been developed to prepare, in high yield, the three derivatives of ganglioside GM3 [α-Neu5Ac-(2-3)-β-Gal-(1-4)-β-Glc-(1-1)-ceramide]: deacetyl-GM3 [α-Neu-(2-3)-β-Gal-(1-4)-β-
Oscar Valiente +4 more
doaj +5 more sources
Monitoring LSO/LYSO Crystal Based Calorimeters [PDF]
Precision light monitoring is important for keeping excellent energy resolution promised by LSO/LYSO crystals in severe radiation environment. In this paper, we report an investigation on the wavelength choice for monitoring LYSO crystal based calorimeters.
F. Yang, Liyuan Zhang, R. Y. Zhu
openalex +3 more sources
Lyso-DGTS Lipid Derivatives Enhance PON1 Activities and Prevent Oxidation of LDL: A Structure–Activity Relationship Study [PDF]
Paraoxonase 1 (PON1) plays a role in regulating reverse cholesterol transport and has antioxidative, anti-inflammatory, antiapoptotic, vasodilative, and antithrombotic activities.
Ali Khattib +4 more
doaj +2 more sources
High Lyso-Gb3 Plasma Levels Associated with Decreased miR-29 and miR-200 Urinary Excretion in Young Non-Albuminuric Male Patient with Classic Fabry Disease [PDF]
Renal involvement is associated with a greater morbidity and mortality in Fabry disease. Pathological albuminuria, the first Fabry nephropathy clinical manifestation, can occur from early childhood, although histological lesions such as tubulo ...
Sebastián Jaurretche +2 more
doaj +2 more sources
Dual-ended readout TOF-DOI PET detectors based on 3.2 mm and 1.6 mm pitch LYSO arrays [PDF]
Background The image quality of positron emission tomography (PET) can be significantly enhanced by using time-of-flight (TOF) and depth-of-interaction (DOI) information.
Haibo Wang +4 more
doaj +2 more sources
A LYSO Calorimeter for the SuperB Factory [PDF]
The SuperB project is an asymmetric e+e? accelerator of 1036cm?2s?1 luminosity, capable of collecting a data sample of 50?75 ab?1 in five years of running. The SuperB electromagnetic calorimeter (EMC), that will be described in this paper, provides energy and direction measurement of photons and electrons, and is used for identification of electrons ...
C. Cecchi +10 more
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Effects of lysophospholipid on utilizing different sources and levels of carbohydrate in rainbow trout (Oncorhynchus mykiss) diet [PDF]
The aim of the present study was to investigate the effects of dietary lysophospholipid and carbohydrates (rye and corn meal) on growth performance, blood and biochemical parameters in rainbow trout.
Batoul Adhami +4 more
doaj +1 more source

