Results 141 to 150 of about 955,547 (405)

Inborn Errors of Metabolism in the Era of Untargeted Metabolomics and Lipidomics. [PDF]

open access: yes, 2019
Inborn errors of metabolism (IEMs) are a group of inherited diseases with variable incidences. IEMs are caused by disrupting enzyme activities in specific metabolic pathways by genetic mutations, either directly or indirectly by cofactor deficiencies ...
Fiehn, Oliver   +2 more
core   +2 more sources

Biodegradable and Recyclable Luminescent Mixed‐Matrix‐Membranes, Hydrogels, and Cryogels based on Nanoscale Metal‐Organic Frameworks and Biopolymers

open access: yesAdvanced Functional Materials, EarlyView.
The study presents biodegradable and recyclable mixed‐matrix membranes (MMMs), hydrogels, and cryogels using luminescent nanoscale metal‐organic frameworks (nMOFs) and biopolymers. These bio‐nMOF‐MMMs combine europium‐based nMOFs as probes for the status of the materials with the biopolymers agar and gelatine and present alternatives to conventional ...
Moritz Maxeiner   +4 more
wiley   +1 more source

Monolysocardiolipin in cultured fibroblasts is a sensitive and specific marker for Barth Syndrome

open access: yesJournal of Lipid Research, 2006
Barth Syndrome (BTHS) is an X-linked recessive disorder that results in abnormal metabolism of the mitochondrial phospholipid cardiolipin (CL). CLs are decreased and monolysocardiolipins (MLCLs), intermediates in CL metabolism, are increased in a variety
Michiel Adriaan van Werkhoven   +3 more
doaj  

Importance of acylcarnitine profile analysis for disorders of lipid metabolism in adolescent patients with recurrent rhabdomyolysis: Report of two cases

open access: yesAnnals of Indian Academy of Neurology, 2014
Metabolic myopathies due to disorders of lipid metabolism are a heterogeneous group of diseases. Newborns may present with hypotonia and convulsions, while progressive proximal muscle weakness or recurrent episodes of muscle weakness accompanied by ...
Yasemin Topçu   +4 more
doaj   +1 more source

Electrothermal supercharging in mass spectrometry and tandem mass spectrometry of native proteins.

open access: yesAnalytical Chemistry, 2013
Electrothermal supercharging of protein ions formed by electrospray ionization from buffered aqueous solutions results in significant increases to both the maximum and average charge states compared to native mass spectrometry in which ions are formed ...
C. Cassou   +3 more
semanticscholar   +1 more source

Demonstration of an All‐Optical AND Gate Mediated by Photochromic Molecules

open access: yesAdvanced Functional Materials, EarlyView.
A logic AND gate that runs on photons is demonstrated. It relies on two spatially separated photochromic molecules that work in tandem. Abstract The realization of a photonic logic AND gate, i.e. a logic AND gate that runs on photons rather than electrons, and where all steps are controlled by light, is demonstrated. In a proof‐of‐principle experiment,
Heyou Zhang   +7 more
wiley   +1 more source

Metabolomics profiling of strawberry (Fragaria x ananassa) F1 population to characterize flavour and nutritional traits [PDF]

open access: yes, 2018
The cultivated strawberry (Fragaria x ananassa) is a highly consumed fruit known for its delicate flavour and nutritional characteristics. However, as fruit quality attributes have been lost after years of traditional breeding, new technological tools ...
Amaya, Iraida   +6 more
core  

Ternary Synergy in Layered Double Hydroxides for Efficient and Stable Nitrate Reduction

open access: yesAdvanced Functional Materials, EarlyView.
Ternary CuZnFe LDH enables efficient electrocatalytic nitrate‐to‐ammonia conversion via controlled in situ reconstruction: zinc leaching creates porous active sites, copper reduced to metallic copper, while iron oxide keeps stable. Synergistic Cu‐Fe redox coupling drives tandem catalysis (nitrate→nitrite→NH3), achieving 95% Faraday efficiency and ...
Jiaqian Kang   +9 more
wiley   +1 more source

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