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Gaucher Disease—Correlation of Lyso-Gb1 with Haematology and Biochemical Parameters [PDF]

open access: goldMetabolites
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
doaj   +3 more sources

Increased Lyso-Gb1 Levels in an Obese Splenectomized Gaucher Disease Type 1 Patient Treated with Eliglustat: Unacknowledged Poor Compliance or Underlying Factors [PDF]

open access: goldMetabolites
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]

open access: goldPLoS ONE, 2011
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]

open access: yesJournal of Lipid Research, 2001
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]

open access: diamondJournal of Physics: Conference Series, 2015
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]

open access: goldAntioxidants, 2022
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]

open access: goldCase Reports in Nephrology, 2019
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]

open access: yesEJNMMI Physics
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]

open access: diamondJournal of Physics: Conference Series, 2011
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
openalex   +3 more sources

Effects of lysophospholipid on utilizing different sources and levels of carbohydrate in rainbow trout (Oncorhynchus mykiss) diet [PDF]

open access: yesتغذیه آبزیان, 2021
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

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