ethylmalonic encephalopathy (ee) is an autosomally recessive inherited disorder with a relentlessly progressive decline in neurological function, usually fatal by the age of ten. it is characterised by generalised hypotonia, psychomotor regression, spastic tetraparesis, dystonia, seizures and, eventually, global neurological failure. approximately 50 reports have been published worldwide descri...

Asymmetric preparation of all-carbon quaternary stereocenters is an important goal. Despite advances in formation of highly enantioenriched products with quaternary stereocenters proximal to a functional group, methods to install quaternary stereocenters isolated from functional groups are limited. Transition metal catalysis offers a potential solution, but prior cross couplings are limited to ...

[l-‘3C]Butyric acid and four different racemic 2-methylbutyric acids labeled with 13C or ‘H at various positions were synthesized to study the mechanism of 2ethylmalonic acid formation as well as several other steps of the R pathway of isoleucine metabolism in rats in uiuo. These are [2-‘HI-, [methyZ-‘3C]-, [methyl-‘HJ-, and 2-methyl[l-‘3C]butyric acid. These labeled precursors were administere...

High-throughput screening for asymmetric catalysts has stimulated an interest in optically-based enantiomeric-excess (ee) sensors, primarily for their improved time and cost efficiency when compared to the standard HPLC analysis. We present herein substituent-effect studies on a recently reported Zn(II) multicomponent assembly that is used for chiral, secondary alcohol ee detemination. The syst...

Achiral meteoritic amino acids, glycine and alpha-methylalanine, with hydrogen isotope (D/H) chirality, acted as the source of chirality in asymmetric autocatalysis with amplification of ee to afford highly enantioenriched 5-pyrimidyl alkanols.

An efficient approach that combines the electrostatically embedded generalized molecular fractionation with conjugate caps (EE-GMFCC) method with conductor-like polarizable continuum model (CPCM), termed EE-GMFCC-CPCM, is developed for ab initio calculation of the electrostatic solvation energy of proteins. Compared with the previous MFCC-CPCM study [Y. Mei, C. G. Ji, and J. Z. H. Zhang, J. Che...

Biphenyl-2,2'-bisfenchol (BIFOL) based chlorophosphite, BIFOP-Cl, exhibits surprisingly high stabilities against hydrolysis as well as hydridic and organometallic nucleophiles. Chloride substitution in BIFOP-Cl proceeds only under drastic conditions. New enantiopure, sterically demanding phosphorus ligands such as a phosphoramidite, a phosphite and a P-H phosphonite (BIFOP-H) are hereby accessi...