Abnormal neurofilament inclusions and segregations in dorsal root ganglia of a Charcot-Marie-Tooth type 2E mouse model

Charcot-Marie-Tooth (CMT) disease or hereditary motor and sensory neuropathy is the most prevalent inherited peripheral neuropathy and is associated with over 90 causative genes. Mutations in neurofilament light polypeptide gene, NEFL cause CMT2E, an axonal form of CMT that results in abnormal structures and/or functions of peripheral axons in spinal cord motor neurons and dorsal root ganglion neurons. We have previously generated and characterized a knock-in mouse model of CMT2E with the N98S mutation in Nefl that presented with multiple inclusions in spinal cord neurons. In this report, we conduct immunofluorescence studies of cultured dorsal root ganglia (DRG) from NeflN98S/+ mice, and show that inclusions found in DRG neurites can occur in embryonic stages. Ultrastructural analyses reveal that the inclusions are disordered neurofilaments packed in high density, segregated from other organelles. Immunochemical studies show decreased NFL protein levels in DRG, cerebellum and spinal cord in NeflN98S/+ mice, and total NFL protein pool is shifted toward the triton-insoluble fraction. Our findings reveal the nature of the inclusions in NeflN98S/+ mice, provide useful information to understand mechanisms of CMT2E disease, and identify DRG from NeflN98S/+ mice as a useful cell line model for therapeutic discoveries

Ocular involvement in Stevens-Johnson syndrome:treatment with amniotic membrane transplantation in the acute phase

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are both part of a spectrum of serious mucocutaneous disorders, most often caused by drugs, with a high morbidity and mortality. In the acute stage, serious skin and mucocutaneous lesions with painful blistering, erosions and systemic involvement present the main focus of attention. The severity of skin manifestations in the acute stage, however, does not necessarily correlate with that of the mucosal lesions. Most feared are long-term sequelae, especially chronic eye involvement, which can be highly disabling. Here, we illustrate the importance of daily evaluation, early recognition and treatment of eye involvement in the acute stage of SJS/TEN, even when skin manifestations and/or initial eye involvement are relatively mild. Timely performed amniotic membrane transplantation in the acute stage can limit irreversible damage, caused by chronic inflammation, and therefore prevent cicatrisation at a later stage.</p

Microbial Community Composition of Two Environmentally Conserved Estuaries in the Midorikawa River and Shirakawa River

To provide a general overview of the microbial communities in environmentally conserved estuaries, the top 5 cm of sediment was sampled from the sandy estuary of the Shirakawa River and from the muddy estuary of the Midorikawa River. Higher amounts of organic matter were detected in the Midorikawa estuary sample than in the Shirakawa estuary sample. Measurement of redox potential revealed that the Shirakawa estuary was aerobic and the Midorikawa estuary was much less aerobic. Clone analysis was performed by targeting partial 16S rRNA gene sequences and using extracted DNA from the samples as a template. Various bacteria were detected, among which Gammaproteobacteria was dominant at both estuaries. Unclassified clones were detected in the Gammaproteobacteria group, mainly among samples from the Midorikawa estuary. Other detected bacterial groups were Alphaproteobacteria, Deltaproteobacteria, Chloroflexi, Actinobacteria, and Bacteroidetes. All the Deltaproteobacteria clones were anaerobic sulfate-reducing bacteria. Those aerobic and anaerobic bacteria coexisted in the top 5 cm of the estuary sediments indicating the surface layer have active sulfur and carbon cycle. Abundance of aerobic Gammaproteobacteria may be an indicator for conserved estuaries

Fluorocarbon adsorption in hierarchical porous frameworks

Metal-organic frameworks comprise an important class of solid-state materials and have potential for many emerging applications such as energy storage, separation, catalysis and bio-medical. Here we report the adsorption behaviour of a series of fluorocarbon derivatives on a set of microporous and hierarchical mesoporous frameworks. The microporous frameworks show a saturation uptake capacity for dichlorodifluoromethane of &#62;4 mmol g-1 at a very low relative saturation pressure (P/Po) of 0.02. In contrast, the mesoporous framework shows an exceptionally high uptake capacity reaching &#62;14 mmol g-1 at P/Poof 0.4. Adsorption affinity in terms of mass loading and isosteric heats of adsorption is found to generally correlate with the polarizability and boiling point of the refrigerant, with dichlorodifluoromethane &#62; chlorodifluoromethane &#62; chlorotrifluoromethane &#62; tetrafluoromethane &#62; methane. These results suggest the possibility of exploiting these sorbents for separation of azeotropic mixtures of fluorocarbons and use in eco-friendly fluorocarbon-based adsorption cooling

Self-Correcting HVAC Controls Project Final Report

This document represents the final project report for the Self-Correcting Heating, Ventilating and Air-Conditioning (HVAC) Controls Project jointly funded by Bonneville Power Administration (BPA) and the U.S. Department of Energy (DOE) Building Technologies Program (BTP). The project, initiated in October 2008, focused on exploratory initial development of self-correcting controls for selected HVAC components in air handlers. This report, along with the companion report documenting the algorithms developed, Self-Correcting HVAC Controls: Algorithms for Sensors and Dampers in Air-Handling Units (Fernandez et al. 2009), document the work performed and results of this project

Feasibility of right-sided total extraperitoneal procedure for inguinal hernia repair after appendectomy: a prospective cohort study

Contains fulltext : 80682.pdf (publisher's version ) (Open Access)BACKGROUND: Totally extraperitoneal (TEP) endoscopic hernia surgery is increasingly popular since it is associated with little postoperative pain and with early return to work. Previous appendectomy may preclude preperitoneal dissection in patients with right-sided hernias. The feasibility of TEP surgery in these patients was the subject of the present study. METHODS: Between January 2005 and February 2007 all consecutive patients undergoing TEP surgery were included in a prospective cohort study. The study group consisted of patients with right-sided and bilateral hernias. Operative times, conversions, complication rates, and return to daily activities were recorded. Patients were divided into two groups according to previous appendectomy. RESULTS: A total of 462 patients with right-sided hernias underwent TEP surgery: 421 patients without previous abdominal surgery (group 1) and 41 patients with previous appendectomy (group 2). The conversion rate was significantly higher in group 2: four patients (10%) were converted to open Lichtenstein repair versus five (1%) in group 1 (p = 0.005). However, we found no significant differences in complication rate, operative time, and return to daily activities. CONCLUSIONS: A right-sided (or bilateral) TEP procedure may be performed safely in patients after previous appendectomy. Despite a higher conversion rate, the vast majority of patients can be operated endoscopically

Grey matter volume alterations in CADASIL: a voxel-based morphometry study

CADASIL is a hereditary disease characterized by cerebral subcortical microangiopathy leading to early onset cerebral strokes and progressive severe cognitive impairment. Until now, only few studies have investigated the extent and localization of grey matter (GM) involvement. The purpose of our study was to evaluate GM volume alterations in CADASIL patients compared to healthy subjects. We also looked for correlations between global and regional white matter (WM) lesion load and GM volume alterations. 14 genetically proved CADASIL patients and 12 healthy subjects were enrolled in our study. Brain MRI (1.5 T) was acquired in all subjects. Optimized-voxel based morphometry method was applied for the comparison of brain volumes between CADASIL patients and controls. Global and lobar WM lesion loads were calculated for each patient and used as covariate-of-interest for regression analyses with SPM-8. Compared to controls, patients showed GM volume reductions in bilateral temporal lobes (p < 0.05; FDR-corrected). Regression analysis in the patient group revealed a correlation between total WM lesion load and temporal GM atrophy (p < 0.05; uncorrected), not between temporal lesion load and GM atrophy. Temporal GM volume reduction was demonstrated in CADASIL patients compared to controls; it was related to WM lesion load involving the whole brain but not to lobar and, specifically, temporal WM lesion load. Complex interactions between sub-cortical and cortical damage should be hypothesized

Aerostructural design optimization of a 100-passenger regional jet with surrogate-based mission analysis

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/106481/1/AIAA2013-4372.pd