Results 91 to 100 of about 22,851 (287)

FENİLKETONÜRİ HASTALARINDA BH4 TEDAVİSİNİN UZUN SÜRELİ İZLEMİ

, 2022
Fenilketonüri (PKU), fenilalanin tirozine dönüşümünü katalize eden fenilalanin hidroksilaz (PAH) enzimini kodlayan gendeki mutasyonların neden olduğu kalıtsal metabolik bozukluktur. Avrupada insidansı 1/10.000 iken Türkiye’de 1/4192 olduğu gösterilmiştir.
Kulu, Bahar, Şahin Uyar, Sibel Burçak, Aydoğan, Ayça, Mintaş, Nuriye Ece, Karalar Pekuz, Özge Kamer, Arslan, Nur   +5 more
core   +3 more sources

Charakterisierung eines Mausmodells zur BH4-sensitiven Phenylketonurie [PDF]

, 2019
Phenylketonurie (PKU) ist eine angeborene Störung des Aminosäurestoffwechsels, die unbehandelt zu schweren neurologischen Schäden führt. In vielen Fällen ist die einzige mögliche Behandlungsoption eine streng eiweißarme Diät. Im Rahmen klinischer Studien
Eichinger, Anna
core   +1 more source

FLP Mediated Conversion of Difluorobenzylalkynes to Fluoroallenyl Oniums via an SN1’ Pathway

Chemistry – A European Journal, EarlyView.
Frustrated Lewis pair (FLP) selective C‒F activation of difluorobenzylalkynes allows the formation of fluoroallenic oniums via an SN1’ substitution pathway. The fluoroallenic onium salts are synthetic precursors that allow subsequent functionalization at allenic and onium positions.
Max Coles, Tianhong Wang, Rubi McLennan, Long Kit Kwan, Bivas Mondal, Chakyu Richard Chan, Rowan D. Young   +6 more
wiley   +1 more source

Reorientational Dynamics in Y(BH4)3·xNH3 (x = 0, 3, and 7) : The Impact of NH3 on BH4- Dynamics

The reorientational dynamics of Y(BH4)3·xNH3 (x = 0, 3, and 7) was studied using quasielastic neutron scattering (QENS) and neutron spin echo (NSE).
Karlsson, M., Andersson, Mikael S., Udovic, Terrence J.,, Karlsson, Maths,, Grinderslev, J. B.,, Udovic, T. J.,, Karlsson, M.,, Faraone, A., Andersson, M. S., Andersson, Mikael S.,, Faraone, A.,, Jensen, T. R.,, Grinderslev, Jakob B.,, Häussermann, Ulrich,, Nagao, M., Andersson, M. S.,, Grinderslev, J. B., Häussermann, U., Udovic, T. J., Jensen, T. R., Nagao, M.,, Häussermann, U.,, Jensen, Torben R.,   +22 more
core   +1 more source

Impact of Ether Side‐Chain Length and Anion Structure on the Physicochemical and Electrochemical Properties of Tetrahydrothiophenium Ionic Liquids

Chemistry – A European Journal, EarlyView.
Tetrahydrothiophenium (Tht) ionic liquids bearing alkyl or ether side chains (one to four oxygen atoms) with FSA or TFSA anions were synthesized. Longer ether chains raised glass transition temperatures and viscosity while lowering conductivity. Short‐ether‐chain ILs, especially FSA‐based ones, exceeded 10−3 S cm−1 at 25°C, comparable to pyrrolidinium ...
Yoshifumi Hirotsu, Yasuyuki Sugimoto, Masahiro Yoshizawa‐Fujita   +2 more
wiley   +1 more source

Melting Behavior and Thermolysis of NaBH4−Mg(BH4)2 and NaBH4−Ca(BH4)2 Composites

, 2015
The physical properties and the hydrogen release of NaBH4–Mg(BH4)2 and NaBH4−Ca(BH4)2 composites are investigated using in situ synchrotron radiation powder X-ray diffraction, thermal analysis and temperature programmed photographic analysis.
Morten B. Ley, Peter M. M. Thygesen, Peter Thygesen, Torben R. Jensen, Morten Ley, Torben Jensen, Elsa Roedern   +6 more
core   +1 more source

Stimulation of Tetrahydrobiopterin Synthesis by 17β-Estradiol in Brain Microvascular Endothelial Cells

Pteridines, 2000
The purpose of this study was to examine whether 17β-estradiol stimulates the synthesis of tetrahydrobiopterin : BH4), which is one of the cofactors of nitric oxide (NO) synthase, in mouse brain microvascular endothelial cells. Addition of 17()-estradiol
Shiota Kazuhiro, Ishii Masakazu, Yamamoto Toshinori, Shimizu Shunichi, Kiuchi Yuji   +4 more
doaj   +1 more source

Lithium ionic conduction in composites of Li(BH4)0.75I0.25 and amorphous 0.75Li2S·0.25P2S5 for battery applications

Electrochimica Acta, 2018
Solid state electrolytic properties of mixtures of Li(BH4)0.75I0.25 and amorphous 0.75Li2S·0.25P2S5 are reported in this study. The enhanced Li-ion conductivities for the Li(BH4)0.75I0.25 phase after addition of 0.75Li2S·0.25P2S5 were found to be within ...
A. El Kharbachi, Yang Hu, K. Yoshida, P. Vajeeston, Sangryun Kim, M. Sørby, S. Orimo, H. Fjellvåg, B. Hauback   +8 more
semanticscholar   +1 more source

Sustainable Hydrogen Production by Glycerol and Monosaccharides Catalytic Acceptorless Dehydrogenation (AD) in Homogeneous Phase

ChemSusChem, Volume 18, Issue 6, March 15, 2025.
Sustainable hydrogen production can be obtained from hydogen‐rich biomass‐derived organic molecules through Acceptorless Dehydrogenation (AD) reactions. Glycerol and sugars are available in large quantities from industry and can be successfully used as substrates in AD protocols, in the presence of tailored, selective, easily tunable and thermally ...
Sylwia Kostera, Luca Gonsalvi
wiley   +1 more source

Dehydrogenation and reaction pathway of Perovskite-Type NH4Ca(BH4)3

, 2018
Perovskite-type borohydride, NH4Ca(BH4)3, is considered as a promising hydrogen storage material due to its high gravimetric hydrogen capacity (15.7 wt%).
Hui Wang, Jia, Yi, Zhu, Min, Yao, Xiangdong, Yi Jia, Jiangwen Liu, Wang, Hui, Xiangdong Yao, Liu, Jiangwen, Ouyang, Liuzhang, Liuzhang Ouyang, Min Zhu, Chengguang Lang, Lang, Chengguang   +13 more
core   +1 more source