Chiral 3,6-dihydroborolo[3,2-]boroles are obtained in one diastereoselective step from the reactions of -(dihaloboryl)arenes with stannole derivatives a complex rearrangement of 1-borolyl-2-(dihaloboryl)arene intermediates.
View Article and Find Full Text PDFPatients heterozygous for germline CBL loss-of-function (LOF) variants can develop myeloid malignancy, autoinflammation, or both, if some or all of their leukocytes become homozygous for these variants through somatic loss of heterozygosity (LOH) via uniparental isodisomy. We observed an upregulation of the inflammatory gene expression signature in whole blood from these patients, mimicking monogenic inborn errors underlying autoinflammation. Remarkably, these patients had constitutively activated monocytes that secreted 10 to 100 times more inflammatory cytokines than those of healthy individuals and CBL LOF heterozygotes without LOH.
View Article and Find Full Text PDFProc Natl Acad Sci U S A
October 2024