γ-rays irradiation effects on dielectric properties of Ti/Au/GaAsN Schottky diodes with 1.2%N

Dielectric properties of As grown and irradiated Ti /Au/GaAsN Schottky diodes with 1.2%N are investigated using capacitance/conductance-voltage measurements in 90–290 K temperature range and 50–2000 kHz frequency range. Extracted parameters are interface state density, series resistance, dielectric constant, dielectric loss, tangent loss and ac conductivity. It is shown that exposure to γ-rays irradiation leads to reduction in effective trap density believed to result from radiation-induced traps annulations. An increase in series resistance is attributed to a net doping reduction. Dielectric constant (ε’) shows usual step-like transitions with corresponding relaxation peaks in dielectric loss. These peaks shift towards lower temperature as frequency decrease. Temperature dependant ac conductivity followed an Arrhenius relation with activation energy of 153 meV in the 200–290 K temperature range witch correspond to As vacancy. The results indicate that γ-rays irradiation improves the dielectric and electrical properties of the diode due to the defect annealing effect

Clinical Heterogeneity of Autosomal Recessive Spastic Paraplegias: Analysis of 106 Patients in 46 Families

Background Hereditary spastic paraplegias (HSPs) are a heterogeneous group of neurodegenerative disorders characterized by progressive and predominant spasticity of the lower limbs, in which dominant, recessive, and X-linked forms have been described. While autosomal dominant HSP has been extensively studied, autosomal recessive HSP is less well known and is considered a rare condition. Objective To analyze the clinical presentation in a large group of patients with autosomal recessive HSP from Portugal and Algeria to define homogeneous groups that could serve as a guide for future molecular studies. Results Clinical features in 106 patients belonging to 46 Portuguese and Algerian families with autosomal recessive HSP are presented, as well as the results of molecular studies in 23 of these families. Five phenotypes are defined: (1) pure early-onset families, (2) pure late-onset families, (3) complex families with mental retardation, (4) complex families with mental retardation and peripheral neuropathy, and (5) complex families with cerebellar ataxia. Six additional families have specific complex presentations, each of which is unique in the present series. Pyramidal signs in the upper limbs and pes cavus are frequent findings, while pseudobulbar signs, including dysarthria, dysphagia, and brisk jaw jerks, are more frequent in the complex forms. The complex forms have a poorer prognosis, while pure forms, particularly those with early onset, are more benign. One Algerian pure early-onset kindred was linked to the locus on chromosome 8, previously reported in 4 Tunisian families. Two of the Portuguese kindreds with complex forms (one with mental retardation and the other associated with hypoplasia of the corpus callosum) showed linkage to the locus recently identified on chromosome 16. Conclusions Although autosomal recessive HSP represents a heterogeneous group of diseases, some phenotypes can be defined by analyzing a large group of patients. The fact that only one Algerian family was linked to chromosome 8 suggests that this is a rare localization even in kindreds with the same ethnic background. Linkage to chromosome 16 was found in 2 clinically diverse Portuguese kindreds, illustrating that this locus is also rare and may correspond to different phenotypes.This study was supported by Généthon, Paris, France; and 2 grants from the Portuguese Foundation for Science and Technology and the Portuguese Health Administration (projects STRDA/C/SAU/277/92 and PECS/C/SAU/219/95)

Effect of Co-60 gamma-ray irradiation on electrical properties of Ti/Au/GaAs1-xNx Schottky diodes

Current-voltage (I-V), capacitance-voltage-frequency (C-V-f) and conductance-voltage-frequency (G/ω-V-f) measurements at room temperature are used to study 50 kGy 60Co γ-ray electrical properties irradiation dependence of Ti/Au/GaAs1−xNx Schottky diodes with 0.2%; 0.4%; 0.8% and 1.2% nitrogen dilution. This γ-ray irradiation induces a permanent damage that has increased ideality factor and series resistance for all samples. It was accompanied by a decrease in Schottky barrier height with nitrogen content up to 0.4%N and remained constant thereafter. Radiation was also found to degrade the reverse leakage current. At high frequency (1 MHz), capacitance and conductance decreased after radiation due to a decrease in net doping concentration. Interface state density and series resistance were determined from C-V-f and G/ω-V-f characteristics using Hill-Coleman methods. Interface states density exponentially decreased with increasing frequency confirming the behavior of interface traps response to ac signal. Series resistance increases after irradiation is attributed to carrier's removal effect and mobility degradation. It has two peaks in the accumulation and inversion region for some diodes (0.4%N, 0.8%N). γ-ray irradiation produced traps levels and recombination centers that reduce relaxation time. An increase in %N content can impede irradiation damage with even some compensation when the percent of diluted nitrogen is high (1.2%N)

Designer exosomes produced by implanted cells intracerebrally deliver therapeutic cargo for Parkinson's disease treatment

Exosomes are cell-derived nanovesicles (50–150 nm), which mediate intercellular communication, and are candidate therapeutic agents. However, inefficiency of exosomal message transfer, such as mRNA, and lack of methods to create designer exosomes have hampered their development into therapeutic interventions. Here, we report a set of EXOsomal transfer into cells (EXOtic) devices that enable efficient, customizable production of designer exosomes in engineered mammalian cells. These genetically encoded devices in exosome producer cells enhance exosome production, specific mRNA packaging, and delivery of the mRNA into the cytosol of target cells, enabling efficient cell-to-cell communication without the need to concentrate exosomes. Further, engineered producer cells implanted in living mice could consistently deliver cargo mRNA to the brain. Therapeutic catalase mRNA delivery by designer exosomes attenuated neurotoxicity and neuroinflammation in in vitro and in vivo models of Parkinson’s disease, indicating the potential usefulness of the EXOtic devices for RNA delivery-based therapeutic applications.ISSN:2041-172

Ataxia with oculomotor apraxia type 2: a clinical and genetic study of 19 patients.

International audienceAtaxia with oculo-motor apraxia type 2 (AOA2) is a recently described autosomal recessive cerebellar ataxia (ARCA) caused by mutations in the senataxin gene (SETX). We analysed the phenotypic spectrum of 19 AOA2 patients with mutations in SETX, which seems to be the third most frequent form of ARCA in Algeria after Freidreich ataxia and Ataxia with vitamin E deficiency. In AOA2 patients, the mean age at onset for all families was in the second decade. Cerebellar ataxia was progressive, slowly leading to disability which was aggravated by axonal polyneuropathy present in almost all the patients. Mean disease duration until wheelchair was around 20 years. Oculo-motor apraxia (OMA) was present in 32% of the patients while convergent strabismus was present in 37%. Strabismus is therefore also very suggestive of AOA2 when associated with ataxia and polyneuropathy even in the absence of OMA. Cerebellar atrophy was more severe in the eldest patients; however it may also be an early sign since it was present in the youngest and paucisymptomatic patients. The initial sign was gait ataxia in all but two patients who presented with head tremor and writer cramp, respectively. Serum alpha-fetoprotein, which was elevated in all tested patients, was a good marker to suggest molecular studies of the SETX gene

Modeling and Simulation Dictionary - English-French-Turkish

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The CHRNE 1293insG founder mutation is a frequent cause of congenital myasthenia in North Africa

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