Neuronal Redox-Imbalance in Rett Syndrome Affects Mitochondria as Well as Cytosol, and Is Accompanied by Intensified Mitochondrial O2 Consumption and ROS Release

In conclusion, we further advanced the molecular understanding of mitochondrial dysfunction in RTT. Intensified mitochondrial O2 consumption, increased mitochondrial ROS generation and disturbed redox balance in mitochondria and cytosol may represent a causal chain, which provokes dysregulated proteins, oxidative tissue damage, and contributes to neuronal network dysfunction in RTT. Introduction Rett syndrome (RTT) is a progressive neurodevelopmental disorder. It primarily affects females, who show the first obvious symptoms within 6–18 months after birth. Among the characteristics are a regression of mental and physical development as well as severe disabilities such as breathing disturbances, loss of speech, autistic features, cognitive impairment, and epilepsy (Rett, 1966; Hagberg et al., 1983; Chahrour and Zoghbi, 2007). In the majority of cases, RTT is caused by spontaneous mutations in the X-chromosomal MECP2 gene encoding for the transcriptional regulator MeCP2 (Amir et al., 1999). Patient and rodent studies confirm that mitochondria are morphologically and functionally affected in RTT (Eeg-Olofsson et al., 1990; Belichenko et al., 2009; Großer et al., 2012; Gold et al., 2014; Park et al., 2014; Valenti et al., 2014; Shulyakova et al., 2017). Human skeletal muscle and frontal lobe biopsy samples revealed swollen mitochondria with vacuolizations and granular inclusions (Ruch et al., 1989; Eeg-Olofsson et al., 1990; Cornford et al., 1994). In male Re...
Source: Frontiers in Physiology - Category: Physiology Source Type: research

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Abbott Laboratories is bringing two of its pediatric devices in the cardiology space to Europe. The Abbott Park, IL-based company has received CE mark for both the Masters HP 15mm rotatable mechanical heart valve and the Amplatzer Piccolo Occluder. The Masters valve is the world's smallest mechanical heart valve and allows doctors to treat babies and toddlers in need of a mitral or aortic heart valve replacement. Until Abbott's device, surgeons could only use a range of larger-sized valves to replace a pediatric heart valve that could not be repaired, which could result in improper fit and complications. The Masters pediat...
Source: MDDI - Category: Medical Devices Authors: Tags: Cardiovascular Source Type: news
CONCLUSION: There is considerable variation in PLLA reconstitution and use. Further well-designed studies are needed to establish the safest, most effective ways to use this product. J Drugs Dermatol. 2019;18(9):880-886. PMID: 31524343 [PubMed - in process]
Source: Journal of Drugs in Dermatology - Category: Dermatology Tags: J Drugs Dermatol Source Type: research
Conclusions: End-of-life decision-making is significantly different for ICU patients with limited English proficiency. Participants identified several barriers and facilitators to high-quality end-of-life decision-making for ICU patients and families with limited English proficiency. Awareness of these factors can facilitate interventions to improve high-quality, compassionate, and culturally sensitive decision-making for patients and families with limited English proficiency.
Source: Critical Care Medicine - Category: Emergency Medicine Tags: Clinical Investigations Source Type: research
Objectives: Electroencephalogram features predict neurologic recovery following cardiac arrest. Recent work has shown that prognostic implications of some key electroencephalogram features change over time. We explore whether time dependence exists for an expanded selection of quantitative electroencephalogram features and whether accounting for this time dependence enables better prognostic predictions. Design: Retrospective. Setting: ICUs at four academic medical centers in the United States. Patients: Comatose patients with acute hypoxic-ischemic encephalopathy. Interventions: None. Measurements and Main ...
Source: Critical Care Medicine - Category: Emergency Medicine Tags: Neurologic Critical Care Source Type: research
Conclusions: Although patients requiring rapid response team activation have high inpatient mortality, most rapid response team activations involve relatively few interventions, which may limit these teams’ ability to improve patient outcomes.
Source: Critical Care Medicine - Category: Emergency Medicine Tags: Feature Articles Source Type: research
Rosenbaum J Abstract PURPOSE OF THE STUDY Lumbar spinal stenosis (LSS) is a serious and fairly frequent disorder with prevalence increasing with age which often results in a disability. The surgical procedures are often very risky due to the greater age of patients with severe stenosis and their comorbidities. The study aimed to assess the functional outcomes and complications in patients who underwent surgical treatment for LSS at one year postoperatively and to identify the differences in the functional outcomes, if any, in dependence on the number of operated segments. MATERIAL AND METHODS It was a prospective ...
Source: Acta Orthopaedica - Category: Orthopaedics Authors: Tags: Acta Chir Orthop Traumatol Cech Source Type: research
Conclusion: More studies need to be conducted on the frequency of all other virulence factors among these isolates, which can provide basic information for improvement or substitution of current vaccinal strains. Overall, as the new designed sets of primers showed more potential in detecting the corresponded genes, researchers can consider them in further studies. PMID: 31523401 [PubMed]
Source: Iranian Journal of Microbiology - Category: Microbiology Tags: Iran J Microbiol Source Type: research
Source: St. Michael's Hospital News and Media - Category: Hospital Management Tags: Hospital News Source Type: news
This study investigated the effects of miR-146a on CSCs' tumorigenesis and invasion. Tumorsphere cells (TCs) were enriched from the HeLa cell line. Real-time PCR, Western blots, ALDH assays, colony formation and invasion assays, luciferase reporter assays and the Xenograft mouse model were used to determine the underlying mechanism of CC. The results showed that TCs displayed higher ALDH activity and miR-146a was upregulated in differentiated TCs. Moreover, miR-146a inhibitor increased colony formation and cell invasion in TCs while miR-146a mimics displayed the opposite roles. An inverse relationship between miR-146a and ...
Source: Artificial Cells, Nanomedicine and Biotechnology - Category: Biotechnology Tags: Artif Cells Nanomed Biotechnol Source Type: research
This study investigated the action model of OH∙ induced by exogenous ferrous iron. Our data showed that ferrous lactate augmented the antibacterial activity of buckwheat honey by producing OH∙ that targeted at components in the respiratory system and the metabolic system such as NADH dehydrogenases and 6-phosphogluconate dehydratases in Pseudomonas aeruginosa. After buckwheat honey was pretreated with ferrous lactate, the activities of NADH dehydrogenases and 6-phosphogluconate showed 79.9% and 99.7% reduction respectively. Activities of these enzymes were much less affected if OH∙ was scavenged by α-tocopherol...
Source: LWT Food Science and Technology - Category: Food Science Source Type: research
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