It is not clear how a highly cationic polymer such as C could cause membrane disruption, which evidently underlies the Catharanthine-hemitartrate antibacterial effects of peptides such as LL-37 and Cecropin A. The observation that diverse nylon-3 polymers display strong growth-inhibitory activity toward E. coli in EZRDM will enable detailed investigations of the mechanism of action via optical imaging methods. Hereditary Inclusion Body Myopathy is a unique autosomal recessive muscle disorder, characterized by adult-onset of muscle weakness in upper and lower limbs. Amazingly, the quadriceps seemed to be spared until late stages of the disease, although recent evidence suggests that rectus femoris could be affected. HIBM has been originally described in Japanese and Iranian Jews but patients are now found worldwide. HIBM muscle fibres present typical pathology, with rimmed vacuoles and cytoplasmic and/or nuclear filamentous inclusions. Intracellular deposits of b-amyloid proteins, altered phosphorylation of tau or activation of the ubiquitin proteasome or of the lysosomal systems can Tenuifolin occasionally be observed. GNE activities rely in two functional domains, controlling respectively the epimerase and the kinase activities. Over 40 different mutations spreading over both domains of Gne can cause HIBM. For example, the most prevalent mutations of Gne gene, M712T and V572L respectively in the Middle Eastern community and in Japanese patients, are both located in the kinase domain of GNE, but other mutations have been identified in its epimerase domain. All these mutations of Gne gene result in different altered enzymatic activities, which should lead to lessened status of cellular sialylation or at least reduced sialylation of specific glycoproteins, such as a-dystroglycan, NCAM, neprilysin or aberrant activity of global systems like lysosomal activity and trafficking. However, the impact of HIBM mutations on the overall sialylation in humans remains unclear.So far, none of these works could identify the molecular pathological mechanism of HIBM and furthermore, a recent model of heterozygous GNE-deficient mice provided clues that cells and organs can manage a certain amount of defects in sialylation, at least down to an overall 25% reduction.