2,4-Dimethyl-N-phenyl­benzene­sulfonamide

The asymmetric unit of the crystal structure of the title compound, C14H15NO2S, contains two mol­ecules. The conformations of the N—C bonds in the C—SO2—NH—C segments of the structure have trans and gauche torsion angles with the S=O bonds. Furthermore, the torsion angles of the C—SO2—NH—C groups in the two mol­ecules are 46.1 (3) (glide image of mol­ecule 1) and 47.7 (3)° (mol­ecule 2). The ortho-methyl groups in the sulfonyl benzene ring are oriented away from the S=O bonds. The two benzene rings are tilted relative to each other by 67.5 (1) and 72.9 (1)° in the two mol­ecules. N—H⋯O and C—H⋯O hydrogen bonds pack the mol­ecules into one-dimensional chains in different directions, resulting in a two-dimensional network

4-Chloro-2-methyl-N-phenyl­benzene­sulfonamide

There are two mol­ecules in the asymmetric unit of the title compound, C13H12ClNO2S, with similar conformations. The orientations of the ortho-methyl groups in the sulfonyl benzene rings are in the direction of the N—H bonds of the sulfonamide groups. In the crystal, the mol­ecules are each linked into centrosymmetric dimers through N—H⋯O hydrogen bonds and packed into a layered structure diagonally in the bc plane

4-Chloro-N-(2-chloro­phen­yl)-2-methyl­benzene­sulfonamide

In the crystal structure of the title compound, C13H11Cl2NO2S, the conformations of the N—C bond in the C—SO2—NH—C segment are trans and gauche with respect to the S=O bonds. The C—S(O2)—N(H)—C torsion angle is 74.8 (4)°, indicating that the mol­ecule is bent at the S atom. In the crystal structure, inversion dimers linked by pairs of N—H⋯O hydrogen bonds occur. An intramolecular N—H⋯Cl inter­action is also present

N-(Phenyl­sulfon­yl)naphtho­[2,1-b]furan-1-carboxamide

In the title compound, C19H13NO4S, the mol­ecule is twisted at the S atom with a C—S—N—C torsion angle of −65.2 (2)° between the benzene ring and the –SO2—NH—C=O segment. The dihedral angle between the benzene and the naphtho­furan ring system is 83.3 (1)°. In the crystal, mol­ecules are linked by N—H⋯O hydrogen bonds into chains running along the c axis. An intra­molecular N—H⋯O(furan) inter­action is also observed

Brown−Vialetto−Van Laere and Fazio−Londe syndromes: SLC52A3 mutations with puzzling phenotypes and inheritance

BACKGROUND: Brown-Vialetto-Van Laere Syndrome (BVVLS) and Fazio-Londe Disease (FLD) are rare neurological disorders presenting with pontobulbar palsy, muscle weakness, and respiratory insufficiency. Mutations in SLC52A2 (hRFVT-2) or SLC52A3 (hRFVT-3) genes can be responsible for these disorders with an autosomal recessive pattern of inheritance. The aim of this study is to screen for mutations in SLC52A2 and SLC52A3 among Indian families diagnosed with BVVLS and FLD. METHODS: SLC52A2 and SLC52A3 were screened in one FLD and three BVVLS patients by exon-specific amplification using PCR and sequencing. In silico predictions using bioinformatics tools and confocal imaging using HEK-293 cells were performed to determine the functional impact of identified mutations. RESULTS: Genetic analysis of a mother and son with BVVLS was identified with a novel homozygous mutation c.710C>T (p.Ala237Val) in SLC52A3. This variant was found to have autosomal pseudo-dominant pattern of inheritance, which was neither listed in the Exome variant server or in 1000 genomes database. In silico analysis and confocal imaging of the p.Ala237Val variant showed higher degree of disorderness in hRFVT3 that could affect riboflavin transport. Furthermore, a common homozygous mutation c.62A>G (p.Asn21Ser) was identified in other BVVLS and FLD patients. Despite having different clinical phenotypes, both BVVLS and FLD disorder can be attributed to this mutation. CONCLUSION: A rare and peculiar pattern of autosomal pseudo-dominant inheritance is observed for the first time in two genetically related BVVLS cases with Indian origin and a common mutation c.62A>G (p.Asn21Ser) in SLC52A3 can be responsible for both BVVLS and FLD with variable phenotypes

Analysis of Distribution Transformer Physiological and Electrical Fault Detection - A Smart Grid Application

  Power grids transport electricity from the point of generation to the market. Power conversion from HV to LV and vice versa occurs in grids, also known as substations. These substations or power grids can be accessible or situated in remote locations. Transformers are used to convert power; they are an essential part of transmission and distribution networks. The method of grid monitoring and maintenance is essentially a very monotonous one. Monitoring the health of the transformers to maintain an uninterrupted power supply to the customers is difficult in such circumstances. Overvoltage, load currents, oil temperature, transformer oil level, and other parameters are monitored. The condition of the distribution transformer’s is evaluated in this article using real-time data from the transformer and specific sensors connected to Raspberry pi and artificial neural networks, are used to analyse the situation and make decisions regarding the health of the transformer. A model has been proposed for continuous monitoring consistent vigilance and swift actions against any faulty situations

Methyl 3,5-dibromo-2-diacetyl­amino­benzoate

The title methyl benzoate compound, C12H11Br2NO4, consists of an ortho-substituted diacetyl­amino group and meta-substituted Br atoms. The crystal packing is stabilized by weak inter­molecular C—H⋯O inter­actions

Evaluation of crop production systems based on locally available biological inputs

Crop production systems that require chemical fertilizers, pesticides, machinery for tillage, and irrigation water are expensive. In countries such as India, they have started to undermine the water security of future generations, contributing to soil and water pollution particularly when synthetic pesticides are not used properly. It is true that agriculture as practiced 100 years ago without modern inputs had lower productivity than present systems of production. However, many premodern practices, such as the use of organic manures to enhance soil fertility and of herbal extracts to protect crops, can be made more efficient by the scientific knowledge that has been gained over the past century, making crop production more sustainable while still achieving high productivity

Novel ALDH3A2 mutations in structural and functional domains of FALDH causing diverse clinical phenotypes in Sjögren-Larsson Syndrome patients

Mutations in ALDH3A2 cause Sjögren-Larsson Syndrome (SLS), a neuro-ichthyotic condition that is caused by deficiency of fatty aldehyde dehydrogenase (FALDH). We screened for novel mutations causing SLS among Indian ethnicity, characterized the identified mutations in silico and in vitro; and retrospectively evaluated their role in phenotypic heterogeneity. Interestingly, asymmetric distribution of non-classical traits was observed in our cases. Nerve conduction studies suggested intrinsic-minus-claw hands in two siblings, a novel neurological phenotype to SLS. Genetic testing revealed 5 novel homozygous ALDH3A2 mutations in six cases: Case-1-NM_000382.2:c.50C>A, NP_000373.1:p.(Ser17Ter); Case-2-NM_000382.2:c.199G>T, NP_000373.1:p.(Glu67Ter); Case-3-NM_000382.2:c.1208G>A, NP_000373.1:p.(Gly403Asp); Case-4-NM_000382.2:c.1325C>T, NP_000373.1:p.(Pro442Leu); Case-5&6-NM_000382.2:c.1349G>A, NP_000373.1:p.(Trp450Ter). The mutations identified were predicted to be pathogenic and disrupts the functional domains of the FALDH. p.(Pro442Leu) at the C-terminal α-helix, might impair substrate gating process. Mammalian expression studies with exon-9 mutants confirmed the profound reduction in the enzyme activity. Diminished aldehyde oxidizing activity was observed with cases-2&3. Cases-2 & 3 showed epidermal hyperplasia with mild intracellular edema, spongiosis, hypergranulosis, and perivascular-interstitial lymphocytic infiltrate and a leaky eosinophilic epidermis. The presence of keratin-milia like lipid vacuoles implies defective lamellar secretion with p.(Gly403Asp). This study improves our understanding of the clinical and mutational diversity in SLS, which might help to fast-track diagnostic and therapeutic interventions of this debilitating disorder. This article is protected by copyright. All rights reserved

First urology simulation boot camp in the United Kingdom

Objective: Simulation is now firmly established in modern surgical training and is applicable not only to acquiring surgical skills but also to non-surgical skills and professionalism. A 5-day intensive Urology Simulation Boot Camp was run to teach emergency procedural skills, clinical reasoning, and communication skills using clinical scenario simulations, endoscopic and laparoscopic trainers. This paper reports the educational value of this first urology boot camp. Subjects and methods: Sixteen urology UK trainees completed pre-course questionnaires on their operative experience and confidence level in common urological procedures. The course included seven modules covering basic scrotal procedures, laparoscopic skills, ureteroscopy, transurethral resection of the prostate and bladder tumour, green light laser prostatectomy, familiarisation with common endoscopic equipment, bladder washout to remove clots, bladder botox injection, setting up urodynamics. Emergency urological conditions were managed using scenarios on SimMan®. The main focus of the course was hands-on training using animal models, bench-top models and virtual reality simulators. Post-course assessment and feedback on the course structure and utility of knowledge gained together with a global outcome score was collected. Results: Overall all the sections of feedback received score of over 4.5/5, with the hands-on training on simulators getting the best score 4.8/5. When trainees were asked “The training has equipped me with enhanced knowledge, understanding and skills,” the average score was 4.9/5.0. The vast majority of participants felt they would recommend the boot camp to future junior trainees. Conclusion: This first UK Urology Simulation Boot Camp has demonstrated feasibility and effectiveness in enhancing trainee’s experience. Given these positive feedbacks there is a good reason to expect that future courses will improve the overall skills of a new urology trainee