نتایج جستجو برای: ethylmalonic encephalopathy

تعداد نتایج: 20072  

2015
Ilaria Pettinati Jürgen Brem Michael A. McDonough Christopher J. Schofield

The ethylmalonic encephalopathy protein 1 (ETHE1) catalyses the oxygen-dependent oxidation of glutathione persulfide (GSSH) to give persulfite and glutathione. Mutations to the hETHE1 gene compromise sulfide metabolism leading to the genetic disease ethylmalonic encephalopathy. hETHE1 is a mono-iron binding member of the metallo-β-lactamase (MBL) fold superfamily. We report crystallographic ana...

2014
Prakash Patel Tarulata Chauhan

Dynamic Source Routing protocol (DSR) has been accepted itself as one of the distinguished and dominant routing protocols for MANETs. From various performance analysis and results, it is shown that DSR has been an outstanding routing protocol that outperforms consistently than any other routing protocols. But it could not pervade the same place when the performance was considered in term of ene...

2006
J. T. Seeman Y. Cai S. Ecklund A. Novokhatski A. Seryi M. Sullivan U. Wienands P. Raimondi

A Super Flavor Factory, an asymmetric energy ee collider with a luminosity of order 10 cms, can provide a sensitive probe of new physics in the flavor sector of the Standard Model. The success of the PEP-II and KEKB asymmetric colliders [1, 2] in producing unprecedented luminosity above 10 cms has taught us about the accelerator physics of asymmetric ee colliders in a new parameter regime. Furt...

Journal: :The Journal of organic chemistry 2008
Xiao-Bing Wang Yong-Gui Zhou

A series of tunable axial chiral bisphosphine ligands have been synthesized from (S)-MeO-Biphep. The Ir complex of the MeO-PEG-supported ligand (S)-4k has been successfully applied in asymmetric hydrogenation of quinolines with up to 92% ee. The catalyst system is air-stable and recyclable.

Journal: :Chemical communications 2005
Luca Bernardi Karl Anker Jørgensen

The direct chiral Lewis acidic enantioselective chlorination and fluorination of beta-keto phosphonates is presented; the chlorination proceeds in high yields and with up to 94% ee using NCS as the chloro source, while the fluorination with (PhSO2)2NF (NFSI) gives the optically active alpha-fluoro-beta-keto phosphonates in moderate to good yields and with up to 91% ee.

Journal: :Neuropediatrics 2007
B Plecko

ganglia, and white matter involvement with brain atrophy in the more progressed state. Chiari I malformation and tethered cord have been described in two siblings [9] but direct association with the primary defect needs proof in further cases. Lactic acid elevation and excretion of ethylmalonic acid have been detected as biochemical markers in the fi rst Italian patients [3] , but in some patie...

Journal: :Cold Spring Harbor perspectives in biology 2013
Valeria Tiranti Massimo Zeviani

Hydrogen sulfide (sulfide, H(2)S) is a colorless, water-soluble gas with a typical smell of rotten eggs. In the past, it has been investigated for its role as a potent toxic gas emanating from sewers and swamps or as a by-product of industrial processes. At high concentrations, H(2)S is a powerful inhibitor of cytochrome c oxidase; in trace amounts, it is an important signaling molecule, like n...

نمودار تعداد نتایج جستجو در هر سال

با کلیک روی نمودار نتایج را به سال انتشار فیلتر کنید