Clustering of ions in cation exchange membranes: a Mössbauer study

A low temperature Mössbauer study of a series of membranes has been made to determine the distribution of ions in them. The presence of three types of iron species has been observed: (1) small units (A) or monomeric species with $\text{d <30}\text{A}\text{?}$; (2) ferric dimers (B) bridged by oxygen or hydroxyl ions; (3) clusters (C) of variable sizes. The percentages of the various species as well as the average size of clusters were evaluated from these spectra.     

Error explanation with distance metrics

In the event that a system does not satisfy a specification, a model checker will typically automatically produce a counterexample trace that shows a particular instance of the undesirable behavior. Unfortunately, the important steps that follow the discovery of a counterexample are generally not automated. The user must first decide if the counterexample shows genuinely erroneous behavior or is an artifact of improper specification or abstraction. In the event that the error is real, there remains the difficult task of understanding the error well enough to isolate and modify the faulty aspects of the system. This paper describes a (semi-)automated approach for assisting users in understanding and isolating errors in ANSI C programs. The approach, derived from Lewis’ counterfactual approach to causality, is based on distance metrics for program executions. Experimental results show that the power of the model checking engine can be used to provide assistance in understanding errors and to isolate faulty portions of the source code.     

On some finite dimensional nonlinear filters for certain diffusions observed in correlated noise

An extension of the one dimensional Bene nonlinear filter to the case of correlated process and observation noises is presented. Conditions are derived so that the optimal nonlinear filter is finite dimensional.     

Bioenergetic and Metabolic Impairments in Induced Pluripotent Stem Cell-Derived Cardiomyocytes Generated from Duchenne Muscular Dystrophy Patients

Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene and dilated cardiomyopathy (DCM) is a major cause of morbidity and mortality in DMD patients. We tested the hypothesis that DCM is caused by metabolic impairments by employing induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs) generated from four DMD patients; an adult male, an adult female, a 7-year-old (7y) male and a 13-year-old (13y) male, all compared to two healthy volunteers. To test the hypothesis, we measured the bioenergetics, metabolomics, electrophysiology, mitochondrial morphology and mitochondrial activity of CMs, using respirometry, LC–MS, patch clamp, electron microscopy (EM) and confocal microscopy methods. We found that: (1) adult DMD CMs exhibited impaired energy metabolism and abnormal mitochondrial structure and function. (2) The 7y CMs demonstrated arrhythmia-free spontaneous firing along with “healthy-like” metabolic status, normal mitochondrial morphology and activity. In contrast, the 13y CMs were mildly arrhythmogenic and showed adult DMD-like bioenergetics deficiencies. (3) In DMD adult CMs, mitochondrial activities were attenuated by 45–48%, whereas the 7y CM activity was similar to that of healthy CMs. (4) In DMD CMs, but not in 7y CMs, there was a 75% decrease in the mitochondrial ATP production rate compared to healthy iPSC-CMs. In summary, DMD iPSC-CMs exhibit bioenergetic and metabolic impairments that are associated with rhythm disturbances corresponding to the patient’s phenotype, thereby constituting novel targets for alleviating cardiomyopathy in DMD patients.