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Defects of the mitochondrial genome are a significant cause of disease. Patients suffer from a wide variety of clinical presentations, ranging from fatal infantile disease to mild muscle weakness. Most disorders, however, are characterized by inexorable progression. As mutations often cause defects in several components of the complexes that couple oxidative phosphorylation, this terminal state of...
The Ugi four component reaction (U-4CR) offers a short and direct route for the synthesis of α-amino acid and peptide derivatives. With 1-amino-5-desoxy-5-thio-2,3,4-O-isobutanoyl-β-d-xylopyranose as a chiral amine component excellent chemical yields and stereoselectivities are obtained. After the U-4CR the chiral auxiliary can be cleaved off selectively under mild conditions. The configuration of...
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