Longitudinal guided waves for monitoring chloride corrosion in reinforcing bars in concrete

Corrosion of reinforcing steel in concrete is one of the major durability problems faced by civil engineers as they maintain an aging infrastructure. The problem accelerates since steel is embedded inside concrete. If it remains unnoticed inside concrete, it further accelerates and can cause loss of life and property. This article discusses a nonintrusive corrosion monitoring technique for early detection of damages in steel embedded in concrete. Corrosion manifests itself in debond and pitting steel bars. Guided ultrasonic waves offer a potentially attractive solution for this problem. But it is imperative to excite the right mode for detection of a particular type of corrosion. In the present work, longitudinal guided ultrasonic waves have been utilized to monitor notch and debond defects in steel bars in concrete simulating pitting and delamination phenomena caused by corrosion. Two ultrasonic techniques of pulse transmission and pulse echo were used to monitor the healthy and damaged specimens. The developed methodology is successfully applied for real time monitoring of RC beam specimens undergoing accelerated chloride corrosion. The ultrasonic signals effectively relate to the state of reinforcing bars

Pathogenic bi-allelic mutations in <em>NDUFAF8 </em>cause leigh syndrome with an isolated complex I deficiency.

Leigh syndrome is one of the most common neurological phenotypes observed in pediatric mitochondrial disease presentations. It is characterized by symmetrical lesions found on neuroimaging in the basal ganglia, thalamus, and brainstem and by a loss of motor skills and delayed developmental milestones. Genetic diagnosis of Leigh syndrome is complicated on account of the vast genetic heterogeneity with &gt;75 candidate disease-associated genes having been reported to date. Candidate genes are still emerging, being identified when "omics" tools (genomics, proteomics, and transcriptomics) are applied to manipulated cell lines and cohorts of clinically characterized individuals who lack a genetic diagnosis. NDUFAF8 is one such protein; it has been found to interact with the well-characterized complex I (CI) assembly factor NDUFAF5 in a large-scale protein-protein interaction screen. Diagnostic next-generation sequencing has identified three unrelated pediatric subjects, each with a clinical diagnosis of Leigh syndrome, who harbor bi-allelic pathogenic variants in NDUFAF8. These variants include a recurrent splicing variant that was initially overlooked due to its deep-intronic location. Subject fibroblasts were found to express a complex I deficiency, and lentiviral transduction with wild-type NDUFAF8-cDNA ameliorated both the assembly defect and the biochemical deficiency. Complexome profiling of subject fibroblasts demonstrated a complex I assembly defect, and the stalled assembly intermediates corroborate the role of NDUFAF8 in early complex I assembly. This report serves to expand the genetic heterogeneity associated with Leigh syndrome and to validate the clinical utility of orphan protein characterization. We also highlight the importance of evaluating intronic sequence when a single, definitively pathogenic variant is identified during diagnostic testing

Revaluation manipulations produce emergence of underselected stimuli following simultaneous discrimination in humans

Stimulus overselectivity occurs when only one of potentially many aspects of the environment controls behaviour. In four experiments, human participants were trained and tested on a trial-and-error simultaneous discrimination task involving two two-element compound stimuli. Overselectivity emerged in all experiments (i.e., one element from the reinforced compound controlled behaviour at the expense of the other). Following revaluation (extinction) of the previously overselected stimulus, behavioural control by the underselected stimulus element emerged without any direct training of that stimulus element. However, while a series of extinction manipulations targeting the revaluation of the overselected stimulus produced differential extinction of that stimulus, they did not result in differential emergence of the previously underselected stimuli. The results are discussed with respect to the theoretical implications for attention-based accounts of overselectivity

<em>LRPPRC</em> mutations cause early-onset multisystem mitochondrial disease outside of the French-Canadian population.

Mitochondrial Complex IV [cytochrome c oxidase (COX)] deficiency is one of the most common respiratory chain defects in humans. The clinical phenotypes associated with COX deficiency include liver disease, cardiomyopathy and Leigh syndrome, a neurodegenerative disorder characterized by bilateral high signal lesions in the brainstem and basal ganglia. COX deficiency can result from mutations affecting many different mitochondrial proteins. The French-Canadian variant of COX-deficient Leigh syndrome is unique to the Saguenay-Lac-Saint-Jean region of Qu&eacute;bec and is caused by a founder mutation in the LRPPRC gene. This encodes the leucine-rich pentatricopeptide repeat domain protein (LRPPRC), which is involved in post-transcriptional regulation of mitochondrial gene expression. Here, we present the clinical and molecular characterization of novel, recessive LRPPRC gene mutations, identified using whole exome and candidate gene sequencing. The 10 patients come from seven unrelated families of UK-Caucasian, UK-Pakistani, UK-Indian, Turkish and Iraqi origin. They resemble the French-Canadian Leigh syndrome patients in having intermittent severe lactic acidosis and early-onset neurodevelopmental problems with episodes of deterioration. In addition, many of our patients have had neonatal cardiomyopathy or congenital malformations, most commonly affecting the heart and the brain. All patients who were tested had isolated COX deficiency in skeletal muscle. Functional characterization of patients&#39; fibroblasts and skeletal muscle homogenates showed decreased levels of mutant LRPPRC protein and impaired Complex IV enzyme activity, associated with abnormal COX assembly and reduced steady-state levels of numerous oxidative phosphorylation subunits. We also identified a Complex I assembly defect in skeletal muscle, indicating different roles for LRPPRC in post-transcriptional regulation of mitochondrial mRNAs between tissues. Patient fibroblasts showed decreased steady-state levels of mitochondrial mRNAs, although the length of poly(A) tails of mitochondrial transcripts were unaffected. Our study identifies LRPPRC as an important disease-causing gene in an early-onset, multisystem and neurological mitochondrial disease, which should be considered as a cause of COX deficiency even in patients originating outside of the French-Canadian population