Response of the topological surface state to surface disorder in TlBiSe2_2

Through a combination of experimental techniques we show that the topmost layer of the topo- logical insulator TlBiSe$_2$ as prepared by cleavage is formed by irregularly shaped Tl islands at cryogenic temperatures and by mobile Tl atoms at room temperature. No trivial surface states are observed in photoemission at low temperatures, which suggests that these islands can not be re- garded as a clear surface termination. The topological surface state is, however, clearly resolved in photoemission experiments. This is interpreted as a direct evidence of its topological self-protection and shows the robust nature of the Dirac cone like surface state. Our results can also help explain the apparent mass acquisition in S-doped TlBiSe$_2$.Comment: 16 pages, 5 figure

Giant axonal neuropathy–associated gigaxonin mutations impair intermediate filament protein degradation

Author Posting. © American Society for Clinical Investigation, 2013. This article is posted here by permission of American Society for Clinical Investigation for personal use, not for redistribution. The definitive version was published in Journal of Clinical Investigation 123 (2013): 1964–1975, doi:10.1172/JCI66387.Giant axonal neuropathy (GAN) is an early-onset neurological disorder caused by mutations in the GAN gene (encoding for gigaxonin), which is predicted to be an E3 ligase adaptor. In GAN, aggregates of intermediate filaments (IFs) represent the main pathological feature detected in neurons and other cell types, including patients’ dermal fibroblasts. The molecular mechanism by which these mutations cause IFs to aggregate is unknown. Using fibroblasts from patients and normal individuals, as well as Gan–/– mice, we demonstrated that gigaxonin was responsible for the degradation of vimentin IFs. Gigaxonin was similarly involved in the degradation of peripherin and neurofilament IF proteins in neurons. Furthermore, proteasome inhibition by MG-132 reversed the clearance of IF proteins in cells overexpressing gigaxonin, demonstrating the involvement of the proteasomal degradation pathway. Together, these findings identify gigaxonin as a major factor in the degradation of cytoskeletal IFs and provide an explanation for IF aggregate accumulation, the subcellular hallmark of this devastating human disease.This work was supported by NIH grants 1P01GM096971 (to R.D. Goldman) and R01 NS062051 (to P. Opal) and a grant from Hannah’s Hope Fund (to R.D. Goldman and P. Opal)

Investigating Environmental Impact Assessment (EIA) In Early Studies (Zero-Phase) to Prevent Delay in Operation of Construction Projects

Delay in project execution is one of the outmost problems that lead to delay to operation and subsequently, early structural erosion or financial supply disturbance, etc. Delay is fallen into several categories and each category has its own specific origin. However, in the present paper, environmental factors causing delay to projects are investigated. Delay to Construction Projects operation can be prevented by embedding environmental studies, namely environmental impact assessment (EIA) in initial studies. Given that environmental events cannot be predicted and we can only rely on statistic reports of previous periods, the effect of embedding such assessment in projects is so useful that every manger is convinced to make use of this assessment in study phase. The research results revealed that it is necessary to present EIA in initial studies to prevent delay to Construction Projects operation. In the following, given to the necessity of checklist, the quality of the designed checklist was investigated. Finally, the checklist was implemented and checked. In this study, each of the research questions was separately tested

Evaluation of Official Governmental (e-Government) Websites for Accessibility in the Middle East

World Wide Web (www), a digital platform providing access to information in an accessible and equitable manner offers immense opportunities to all categories of users. Its inclusivism enables people with physical challenges to access websites, identify, appreciate, navigate, utilize, interact and contribute to the web. This study evaluates the web accessibility features of 58 governmental websites of 17 countries in the Middle East by means of the manual and the automated testing methods with different benchmarks based on the Web Content Accessibility Guidelines 2.0 (WCAG) and Section 508 standards for the website accessibility, for each to achieve a more comprehensive and efficient result for better analysis. The results of the findings are contrasting; from the manual evaluation based on specific criteria, countries such as United Arab Emirates, Saudi Arabia, Bahrain and Oman scored high percentages in majority of the criteria but scored very low in the automated evaluation

Novel homozygous missense mutation in GAN associated with Charcot-Marie-Tooth disease type 2 in a large consanguineous family from Israel.

BACKGROUND: CMT-2 is a clinically and genetically heterogeneous group of peripheral axonal neuropathies characterized by slowly progressive weakness and atrophy of distal limb muscles resulting from length-dependent motor and sensory neurodegeneration. Classical giant axonal neuropathy (GAN) is an autosomal recessively inherited progressive neurodegenerative disorder of the peripheral and central nervous systems, typically diagnosed in early childhood and resulting in death by the end of the third decade. Distinctive phenotypic features are the presence of "kinky" hair and long eyelashes. The genetic basis of the disease has been well established, with over 40 associated mutations identified in the gene GAN, encoding the BTB-KELCH protein gigaxonin, involved in intermediate filament regulation. METHODS: An Illumina Human CytoSNP-12 array followed by whole exome sequence analysis was used to identify the disease associated gene mutation in a large consanguineous family diagnosed with Charcot-Marie-Tooth disease type 2 (CMT-2) from which all but one affected member had straight hair. RESULTS: Here we report the identification of a novel GAN missense mutation underlying the CMT-2 phenotype observed in this family. Although milder forms of GAN, with and without the presence of kinky hair have been reported previously, a phenotype distinct from that was investigated in this study. All family members lacked common features of GAN, including ataxia, nystagmus, intellectual disability, seizures, and central nervous system involvement. CONCLUSIONS: Our findings broaden the spectrum of phenotypes associated with GAN mutations and emphasize a need to proceed with caution when providing families with diagnostic or prognostic information based on either clinical or genetic findings alone

Evaluation of hyperglycaemic risk of atorvastatin: a dose dependent study on hyperlipidaemic rats

Background: Statins (β-hydroxy β-methylglutaryl-CoA (HMG-CoA) reductase inhibitors) are the most prescribed medications worldwide to treat hyperlipidaemia with a proven ability to reduce major cardiovascular events. Recent data have revealed that statin therapy is associated with an increased risk for developing diabetes. The risk was most significant in patients taking atorvastatin, rosuvastatin and simvastatin.Methods: Rats were divided into 3 groups, each comprising of 6 rats. Hyperlipidaemia was induced in all the animals after feeding with high fat diet for 15 days. Rats of groups 1, 2 and 3 were given atorvastatin 1.8 mg/kg (low-dose), 3.6 mg/kg (moderate-dose) and 7.2 mg/kg (intensive-dose) respectively orally for 60 days. 12 hours fasted blood samples were collected and analyzed for serum lipid profile, fasting blood glucose and HbA1c levels.Results: The percentage increase in plasma blood glucose after 60 days of treatment in groups 1, 2, and 3 is 29.93%, 60.03% and 72.42% respectively and the variation in all the groups is statistically significant, p<0.0001. Regarding HbA1c values, the variation in low-dose group is statistically insignificant whereas the percentage increase in moderate-dose and intensive-dose groups is 19.45% (p<0.001) and 43.37% (p<0.0001) respectively.Conclusions: In conclusion, there is significant increase in blood glucose and HbA1c levels leading to new-onset diabetes in both moderate-dose and intensive-dose groups. The risk is more in intensive-dose group when compared to moderate-dose group

Comparative study of lateral entry versus crossed entry pinning for paediatric supracondylar humerus fractures: a case series

Closed reduction and percutaneous pin fixation techniques have been proposed as treatment strategies for displaced supracondylar humeral fractures (SCHFs) in children. Commonly lateral pinning and cross pinning techniques are utilised for fixation. However, controversy exists regarding the selection of the appropriate procedure. A prospective study with 24 cases of displaced fracture supracondylar humerus, treated by lateral pinning and cross pinning, was conducted between August 2022 and May 2024 at Department of Orthopaedics at J.J.M. Medical College, Davangere. Patients were treated with either the lateral entry pin alone or the cross pinning with a combination of lateral entry pin and medial entry pin. Age, gender, fractured side, duration of surgery, postoperative complications, surgical approach, direction of pin application (lateral or cross), and Modified Flynn grading system grade was noted for study outcome. No difference was found between lateral pinning and crossed pinning groups in terms of the grade of the modified Flynn grading system and complications like iatrogenic ulnar nerve damage, loss of reduction. Pin tract infection was seen in one patient

High performance DIF-FFT using dissimilar partitioned LUT based Distributed Arithmetic

Real-time data processing systems utilize Digital Signal Processing (DSP) functions as the base modules. Most of the DSP functions involve the implementation of Fast Fourier Transform (FFT) to convert the signals from one domain to another domain. The major bottleneck of Decimation in frequency-Fast Fourier Transform (DIF-FFT) implementation lies in using a number of Multipliers. Distributed arithmetic (DA) is considered as one of the efficient techniques to implement DIF-FFT. In this approach, the multipliers are not used. The proposed technique exploits the very advantage of the look-up table by storing the Twiddle factors, thereby avoiding the multipliers required in the butterfly structure. DIF-FFT using Distributed Arithmetic (DIF-FFT DA) models, with different adders such as Ripple carry adder (RCA), Carry-lookahead adder (CLA), and Sklansky prefix graph adder, are proposed in this paper. The three proposed models are synthesized using Cadence 6.1 EDA tools with a 45nm CMOS technology. Compared to the traditional method, it is observed that the area is improved by 53.11%, 53.35%, and 50.15%, power is improved by 42.31%, 42.52%, and 40.39%, and delay is improved by 45.26%, 45.42%, 41.80%, respectively

Medial patellofemoral ligament reconstruction for patellofemoral instability: a new surgical technique

The purpose of this study was to present new surgical technique for MPFL reconstruction. We also describe its functional outcome, complications, and the advantages of the procedure. This study is a prospective analysis of collected data during the period of august 2018 to January 2020. Ten cases of patients with recurrent symptomatic patellar instability and who underwent isolated MPFL reconstruction were included in the study. Kujala scoring and lysholm scoring was done to assess the functional outcome at follow-up. Post-operative dislocation and apprehension were recorded in each case along with any complication. Pre-operative Kujala score was 36.80 which improved to 89.80 postoperatively at the time follow-up. Pre-operative lysholm score was 36.80 which improved to 92.70 postoperatively at the time follow-up. The improvement in Kujala score and Lysholm score was found to be highly significant (p&lt;0.01). We have done a simple technique where MPFL is reconstructed anatomically to restore kinematics and stability. Consistent good results with early rehabilitation can be obtained using the described technique