New and Efficient Chiral Selenium Electrophiles

New chiral diselenides were prepared in a few steps from readily available starting materials. The selenium electrophiles generated from these diselenides were used for the efficient stereoselective inter- and intramolecular functionalization of alkenes. The substitution pattern influences the stereoselectivities and protection of the hydroxy moiety in the chiral side chain led to increased selectivities and yields in the selenenylation reactions. An additional substituent in the second ortho position was advantageous as well. Addition products with up to 96 % de were obtained. The influence of the nucleophile on the outcome of selenenylations of alkenes was studied to some extent as well

Theoretical investigations on the stereoselective selenenylation reaction of alkenes

Ab initio calculations have been performed to study the stereoselective selenenylation reaction of alkenes with chiral selenium electrophiles. The interaction of the heteroatom of the chiral side chain with the selenium atom in the reagent has been investigated by a detailed conformational analysis. Calculations of the seleniranium intermediates with different substituted alkenes have been performed as well. The reversal of selectivity changing from aromatic to aliphatic alkenes as substrates in the selenenylation reaction has already been proven by experiment and is now supported by the calculations described herein

Mutations in ZBTB20 cause Primrose syndrome

Primrose syndrome and 3q13.31 microdeletion syndrome are clinically related disorders characterized by tall stature, macrocephaly, intellectual disability, disturbed behavior and unusual facial features, with diabetes, deafness, progressive muscle wasting and ectopic calcifications specifically occurring in the former. We report that missense mutations in ZBTB20, residing within the 3q13.31 microdeletion syndrome critical region, underlie Primrose syndrome. This finding establishes a genetic link between these disorders and delineates the impact of ZBTB20 dysregulation on development, growth and metabolism