gebhart/patrepeval huggingface dataset:
Human keratin diseases: (1996)

Keratins are heteropolymeric proteins which form the intermediate filament cytoskeleton in epithelial cells. Since 1991, mutations in several keratin genes have been found to cause a variety of human diseases affecting the epidermis and other epithelial structures. Epidermolysis bullosa simplex (EBS) was the first mechanobullous disease for which the underlying genetic lesion was found, with mutations in both the K5 and K14 genes rendering basal epidermal keratinocytes less resilient to trauma, resulting in skin fragility. The site of mutation in the keratin protein correlates with phenotypic severity in this disorder. Since mutations were identified in the basal cell keratins, the total number of keratin genes associated with diseases has risen to eleven. The rod domains of suprabasal keratins K1 and K10 are mutated in bullous congenital ichthyosiform erythroderma (BCIE; also called epidermolytic hyperkeratosis, EH) and mosaicism for K1/K10 mutations results in a nevoid distribution of EH. An unusual mutation in the VI domain of K1 has also been found to cause diffuse non-epidermolytic palmoplantar keratoderma (DNEPPK). Mutations in palmoplantar specific keratin K9 cause epidermolytic palmoplantar keratoderma (EPPK) and mutations in the late differentiation suprabasal keratin K2e cause ichthyosis bullosa of Siemens (IBS). In the last year or so, mutations were discovered in differentiation specific keratins K6a and K16 causing pachyonychia congenita type 1 and K17 mutations occur in pachyonychia congenita type 2. K16 and K17 mutations have also been reported to produce phenotypes with little or no nail changes: K16 mutations can present as focal non-epidermolytic palmoplantar keratoderma (NEPPK) and K17 mutations can result in a phenotype resembling steatocystoma multiplex. Recently, mutation of mucosal keratin pair K4 and K13 has been shown to underlie white sponge nevus (WSN). This year, the first mutations in a keratin-associated protein, plectin, were shown to cause a variant of epidermolysis bullosa associated with late-onset muscular dystrophy (MD-EBS). An unusual mutation has been identified in K5 which is responsible for EBS with mottled pigmentation and genetic linkage analysis suggests that the hair disorder monilethrix is likely to be due to a mutation in a hair keratin. The study of keratin diseases has led to a better understanding of the importance of the intermediate filament cytoskeleton and associated connector molecules in maintaining the structural integrity of the epidermis and other high stress epithelial tissues, as well as allowing diagnosis at the molecular level thus facilitating prenatal testing for this heterogeneous group of genodermatoses.
Interfacial tension minima in oil–water–surfactant systems. Effects of alkane chain length and presence of n-alkanols in systems containing aerosol OT (1986)

In alkane and aqueous NaCl systems containing Aerosol OT (AOT) the alkane–solution interfacial tension becomes constant at the onset of surfactant aggregation, which can occur in either the aqueous or alkane phase, depending on conditions. The constant tension, γc, can attain ultralow values (ca. 10–3 mN m–1), and has been shown previously to pass through a minimum value with respect to salt concentration and temperature. In the present paper we report an investigation into minima in γc brought about by variation of the chain length of the alkane, and by addition of n-alkanols (‘cosurfactants’) to the system. Results are discussed in terms of the effective molecular geometry of the surfactant and cosurfactant, and penetration of alkane into the surfactant monolayer. A thermodynamic treatment of the effect of cosurfactant demonstrates that minimum γc results when the ratio of surfactant to cosurfactant is equal at the plane alkane–water interface and in mixed aggregates, as expected from the simple geometrical picture.
Decorrelating decision-feedback multiuser detector for synchronous code-division multiple-access channel (1993)

A decorrelating decision-feedback detector (DF) for synchronous code-division multiple-access (CDMA) that uses decisions of the stronger users when forming decisions for the weaker ones is described. The complexity of the DF is linear in the number of users, and it requires only one decision per user. It is shown that performance gains with respect to the linear decorrelating detector are more significant for relatively weak users and that the error probability of the weakest user approaches the single-user bound as interferers grow stronger. The error rate of the DF is compared to those of the decorrelator and the two-stage detector.< <ETX xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink">&gt;</ETX>
Nature of the carbonium ion. VII. Dehydronorbornyl cations from thiocyanate isomerizations (1971)

ADVERTISEMENT RETURN TO ISSUEPREVArticleNEXTNature of the carbonium ion. VII. Dehydronorbornyl cations from thiocyanate isomerizationsLangley A. Spurlock and Walter G. CoxCite this: J. Am. Chem. Soc. 1971, 93, 1, 146–151Publication Date (Print):January 1, 1971Publication History Published online1 May 2002Published inissue 1 January 1971https://pubs.acs.org/doi/10.1021/ja00730a026https://doi.org/10.1021/ja00730a026research-articleACS PublicationsRequest reuse permissionsArticle Views40Altmetric-Citations8LEARN ABOUT THESE METRICSArticle Views are the COUNTER-compliant sum of full text article downloads since November 2008 (both PDF and HTML) across all institutions and individuals. These metrics are regularly updated to reflect usage leading up to the last few days.Citations are the number of other articles citing this article, calculated by Crossref and updated daily. Find more information about Crossref citation counts.The Altmetric Attention Score is a quantitative measure of the attention that a research article has received online. Clicking on the donut icon will load a page at altmetric.com with additional details about the score and the social media presence for the given article. Find more information on the Altmetric Attention Score and how the score is calculated. Share Add toView InAdd Full Text with ReferenceAdd Description ExportRISCitationCitation and abstractCitation and referencesMore Options Share onFacebookTwitterWechatLinked InRedditEmail Other access optionsGet e-Alertsclose Get e-Alerts
Stretched call model for next generation cellular networks (2004)

This paper examines the possibility of implementation of relaying in 3G cellular systems by allowing calls between a mobile terminal and base station to be forwarded by some other mobile device in the cell. This "stretched" call provides improvements in throughput, capacity, energy savings and coverage. The main contribution of this paper is to enumerate the problems that are introduced due to relaying, and how they could be resolved. We consider cdma2000, UTRA-FDD and UTRA-TDD technologies of 3G cellular, and discuss stretched models suitable for best throughput and delay metrics.
Sub-60mV-swing negative-capacitance FinFET without hysteresis (2015)

In this work, we report the first Negative-Capacitance FinFET. ALD Hf042Zr058O2 is added on top of the FinFET's gate stack. The test devices have a floating internal gate that can be electrically probed. Direct measurement found the small-signal voltage amplified by 1.6X maximum at the internal gate in agreement with the improvement of the subthreshold swing (from 87 to 55mV/decade). ION increased by >25% for the IOFF. For the first time, we demonstrate that raising HfZrO2 ferroelectricity, by annealing at higher temperature, reduces and eliminates IV hysteresis and increases the voltage gain. These discoveries will guide future theoretical and experimental work.
The role of malic enzyme in the regulation of lipid accumulation in filamentous fungi (1999)

The hypothesis is advanced that NADP(+)-malic enzyme (ME; EC 1.1.1.40) is an important activity in regulating the extent of lipid accumulation in filamentous fungi. In Mucor circinelloides, a fungus capable of accumulating only 25% (w/w, dry wt) lipid, even under the most propitious conditions, ME disappears 15-20 h after nitrogen exhaustion, coincident with the cessation of lipid accumulation. In contrast, ME in Mortierella alpina, a fungus capable of accumulating 50% (w/w, dry wt) lipid, remains active for over 60 h after N-exhaustion during which time lipid accumulation continues. No other enzyme activity studied, including the lipogenic enzymes acetyl-CoA carboxylase, fatty acid synthase, diacyglycerol acyltransferase, ATP: citrate lyase and the NADPH-generating enzymes glucose-6-phosphate dehydrogenase, 6-phosphogluconate dehydrogenase and NADP+:isocitrate dehydrogenase, demonstrated any correlation with the accumulation of storage lipid in either fungus. Full activity of ME is restored in Mr. circinelloides within 4 h by adding NH4+ to the cultures, but this is prevented by adding cycloheximide as an inhibitor of protein synthesis. This suggests that the decrease in ME activity occurs due to down-regulation of the ME gene.
Exposure schedule for multiplexing holograms in photopolymer films (1996)

An iterative method is introduced for determining the exposure schedule for multiplexing holograms in saturable recording materials, such as photopolymers. This method is designed to share all or part of the available dynamic range of the recording material among the holograms to be multiplexed. Using exposure schedules derived from this method, the authors find that the diffraction efficiency of DuPont's HRF-150 38-and 100-m photopolymer scale is (2.2/M) 2 and (6.5/M) 2 respectively, where M is the number of holograms recorded. Finally, 1000 holograms were multiplexed at a single location in the 100-m thick photopolymer using an exposure schedule derived with this method.
Aberrant Expression of Gangliosides in Human Renal Cell Carcinomas (1993)

Aberrant and elevated ganglioside expression has been observed in neoplasms, and has been shown to be an important marker of tumor progression. We therefore studied the gangliosides of renal cell carcinoma (RCC) by analyzing gangliosides from 18 RCC biopsies, 10 RCC lines and 5 normal kidney biopsies. A comparison of tumor with normal tissue revealed a significant difference in individual ganglioside expression in which the former consistently expressed eight major gangliosides, GM3, GM2, GM1, GD3, GD1A, GD2, GD1B and GT1B, according to the nomenclature of Svennerholm. There was a notable significant mean increase in the expression of GM2, GM1 and GD1A and a significant decrease in the expression of GD3 in tumor tissue compared with normal kidney tissue. Compared with tumor biopsy tissue, RCC cell lines showed a significant decrease in the expression of GM3, but a significant increase in GM2, GM1 and GD2. There was a marked increase in a pathway gangliosides (GM2, GM1, and GD1a) in RCC biopsies and cell lines compared with normal kidney. These studies indicating that RCC have markedly aberrant ganglioside expression similar to neural origin tumors may relate to the activation of a ganglioside pathway enzymes. Gangliosides expressed on RCC tumors may be important markers of tumor progression and target antigens for immunotherapy.
The relative contribution of the CD28 and gp39 costimulatory pathways in the clonal expansion and pathogenic acquisition of self-reactive T cells. (1996)

The zona pellucida (ZP), an ovarian extracellular structure, contains three major glycoproteins: ZP1, ZP2, and ZP3. A ZP3 peptide contains both an autoimmune oophoritis-inducing T cell epitope and a B cell epitope that induces autoantibody to ZP. This study investigates two major T cell costimulation pathways in this disease model. Herein we show that blockage of glycoprotein (gp)39 and CD40 interaction with gp39 monoclonal antibody (mAb) results in the failure to induce both autoimmune oophoritis and autoantibody production. Inhibition of ligand binding to the CD28 receptor with the fusion protein, murine CTLA4-immunoglobulin (Ig), also results in failure to generate antibody to ZP and significantly reduces disease severity and prevalence. Surprisingly, the frequencies of antigen-specific T cells in anti-gp39 mAb-treated mice, CTLA4-Ig treated mice, and in mice given control hamster IgG or control fusion protein L6, were equivalent as determined by limiting dilution analysis (approximately equals 1:5,000). These T cells, which produced comparable amounts of interleukin 4 and interferon gamma in vitro, were able to transfer oophoritis to normal recipients. When anti-gp39 mAb and CTLA4-Ig were given together, the effect was additive, leading to inhibition of T cell activation as determined by in vitro proliferation and limiting dilution analysis (approximately equals 1:190,000); disease and antibody responses were absent in these mice. By studying these two costimulatory pathways in parallel, we have shown that autoimmune disease and autoantibody production are inhibitable by blocking either the gp39 or the CD28 pathway, whereas inhibition of clonal expansion of the effector T cell population occurs only when both pathways are blocked.
Silicon Surface Structure-Controlled Oleophobicity (2010)

Superoleophobic surfaces display contact angles >150 degrees with liquids that have lower surface energies than does water. The design of superoleophobic surfaces requires an understanding of the effect of the geometrical shape of etched silicon surfaces on the contact angle and hysteresis observed when different liquids are brought into contact with these surfaces. This study used liquid-based metal-assisted etching and various silane treatments to create superoleophobic surfaces on a Si(111) surface. Etch conditions such as the etch time and etch solution concentration played critical roles in establishing the oleophobicity of Si(111). When compared to Young's contact angle, the apparent contact angle showed a transition from a Cassie to a Wenzel state for low-surface-energy liquids as different silane treatments were applied to the silicon surface. These results demonstrated the relationship between the re-entrant angle of etched surface structures and the contact angle transition between Cassie and Wenzel behavior on etched Si(111) surfaces.
Stereocontrolled Total Syntheses of Shark Cartilage Chondroitin Sulfate D-Related Tetra- and Hexasaccharide Methyl Glycosides (2002)

Expeditious and stereocontrolled syntheses of β-D-GlcpA(2SO4)-(1⇄3)-[β-D-GalpNAc(6-SO4)-(1⇄4)-β-D-GlcpA(2-SO4)(1⇄3)]n-β-D-GalpNAc(6-SO4)-(1⇄OMe) (where n = 1 and 2), which represent structural elements of shark cartilage chondroitin sulfate D, are reported for the first time. The compounds were obtained from a common key disaccharide donor 15, which was used in an iterative way, and in which the 2-deoxy-2-trichloroacetamido group was used as an efficient stereocontrolling auxiliary. The D-glucuronyl donor 7 was easily prepared from D-glucose, whereas the D-galactosaminyl acceptor 11 was synthesized starting from D-glucosamine precursors. (© Wiley-VCH Verlag GmbH, 69451 Weinheim, 2002)
Modulation of antibody affinity by a non‐contact residue (1993)

Antibody LB4, produced by a spontaneous variant of the murine anti-digoxin monoclonal antibody 26-10, has an affinity for digoxin two orders of magnitude lower than that of the parent antibody due to replacement of serine with phenylalanine at position 52 of the heavy chain variable region (Schildbach, J.F., Panka, D.J., Parks, D.R., et al., 1991, J. Biol. Chem. 266, 4640-4647). To examine the basis for the decreased affinity, a panel of engineered antibodies with substitutions at position 52 was created, and their affinities for digoxin were measured. The antibody affinities decreased concomitantly with increasing size of the substituted side chains, although the shape of the side chains also influenced affinity. The crystal structure of the 26-10 Fab complexed with digoxin (P.D.J., R.K. Strong, L.C. Sieker, C. Chang, R.L. Campbell, G.A. Petsko, E.H., M.N.M., & S.S., submitted for publication) shows that the serine at heavy chain position 52 is not in contact with hapten, but is adjacent to a tyrosine at heavy chain position 33 that is a contact residue. The mutant antibodies were modeled by applying a conformational search procedure to position side chains, using the 26-10 Fab crystal structure as a starting point. The results suggest that each of the substituted side chains may be accommodated within the antibody without substantial structural rearrangement, and that none of these substituted side chains are able to contact hapten. These modeling results are consistent with the substituents at position 52 having only an indirect influence upon antibody affinity.(ABSTRACT TRUNCATED AT 250 WORDS)
An autologous fibrinogen‐based adhesive for use in otologic surgery (1988)

Successful middle ear surgery requires the availability of a safe, effective bonding material. Side effects caused by synthetic materials have led to the use of biologic adhesives; however, they carry the risk of transmission of infectious disease if they are prepared from pooled human blood. A procedure for the production of an autologous fibrinogen-based adhesive using polyethylene glycol to precipitate the fibrinogen-factor XIII component from plasma is described. This procedure requires 40 ml of whole blood and approximately 3 hours' preparation time, and it can be performed in any blood bank with the facilities for sterile techniques. This adhesive has been used successfully for otologic surgery in 12 patients, and further study of the use of this biologic adhesive for other microsurgical techniques should be undertaken.
Mapping the Pupil-Glint Vector to Gaze Coordinates in a Simple Video-Based Eye Tracker (2013)

In a video-based eye tracker, the normalized pupil-glint vector changes as the eyes move. Using an appropriate model, the pupil-glint vector can be mapped to gaze coordinates. Using a simple hardware configuration with one camera and one infrared source, several mapping functions – some from literature and some derived here – were compared with one another with respect to the accuracy that could be achieved. The study served to confirm the results of a previous study with another data set and to expand on the possibilities that are considered from the previous study. The data of various participants was examined for trends which led to derivation of a mapping model that proved to be more accurate than all but one model from literature. It was also shown that the best calibration configuration for this hardware setup is one that contains fourteen targets while taking about 20 seconds for the procedure to be completed.
Programmable high-amplitude balanced stimulus current-source for implantable microstimulators (2002)

In this paper, a 5 bit programmable current-source dedicated to implantable microstimulators is presented. Generating a maximum current of 4 mA through a 1 K/spl Omega/ load, it takes advantage of the BiCMOS 0.8 /spl mu/m technology which allows high current-level output-stage. It occupies a silicium area smaller than 0.026 mm/sup 2/. Simulation results showed good linearity and low power consumption. Its architecture allows the generation of bi-directional current pulses and features an almost null DC offset. Finally, the proposed current-source can be easily modified to output a smaller current range, enabling greater precision and giving the possibility to stimulate nerves with higher impedances.
Formation of a Biologically Active Toxin Complex of the Binary Clostridium botulinum C2 Toxin without Cell Membrane Interaction (2006)

Clostridium botulinum produces a binary toxin, which is composed of two separate proteins. The enzyme component, C2I, is an ADP-ribosyltransferase which modifies G-actin of eukaryotic cells. The proteolytically activated binding/translocation component, C2IIa, forms ring-shaped heptamers, which bind to cell receptors and mediate the transport of C2I into the cytosol of target cells. According to the current model, receptor-bound C2IIa serves as a docking platform for C2I on the cell surface. Following assembly of C2I, the toxin complex is taken up via receptor-mediated endocytosis, and finally, C2IIa facilitates translocation of C2I from acidic endosomes into the cytosol. Our data support an alternative scenario for the early steps of interaction of the C2 toxin and eukaryotic cells, due to the fact that C2IIa and C2I can interact prior to binding of the toxin to the cell surface. The C2IIa-C2I complex, which was formed in a cell-free system, was detected by native gel electrophoresis and subsequent immunoblot analysis or radiolabeling methods. The preformed C2 toxin complex ADP-ribosylated actin in vitro and induced cell rounding. The interaction of C2I with C2IIa did not enhance the binding of C2IIa to the cellular receptor. Intoxication of Vero cells and of human colon carcinoma cells (CaCo-2) was significantly enhanced when the preformed toxin complex was added to cultured cells as compared to addition of the single components.
Preclinical and clinical progress in hemophilia gene therapy (2010)

New insights from clinical trials and advances in preclinical studies may ultimately pave the way toward a cure in patients suffering from hemophilia.
Electron transfer during metal-assisted and stain etching of silicon (2015)

The etching of silicon in fluoride solutions is limited by the kinetics of charge transfer not thermodynamics. This characteristic is what gives fluoride etching its great versatility in making different types of nanostructures as the result of self-limiting chemistry. This review approaches the kinetics of electron transfer from silicon and metal-coated silicon to a solution phase species from a fundamental point of view in order to establish a better understanding of the mechanisms of nanostructure formation during metal-assisted and stain etching of silicon. Band bending calculations demonstrate that diffusion of holes away from low work function metals such as Ag is not possible. Similarly diffusion of holes outside of the space charge layer is not possible for high work function metals such as Au, Pd and Pt. While direct hole injection may be important for etch track pore formation in the immediate vicinity of the metal, the charge imbalance on or near the metal causes the metal to act like a nanopower supply that polarizes the surrounding Si. This second mechanism is implicated in nonlocal etching of Si during metal-assisted etching.
Cryocrystallography of ribosomal particles (1989)

Crystals suitable for X-ray study have been prepared from biochemically active ribosome particles or their complexes with tRNA and polypeptide chains. At ambient temperature the useful lifetime of these crystals under synchrotron irradiation is limited to a few minutes. However, upon cooling to cryogenic temperatures around 85 K, the original resolution limit (up to 4.5 A) can be recorded and radiation damage is virtually eliminated. Hence it has become possible to collect a complete data set from one single crystal. Crystals were cooled as rapidly as possible, either in a cold gas stream, or by immersion in liquid propane. Before cooling crystals were transferred either to an inert hydrocarbon environment, or to solutions similar to the crystallizing ones but with a higher viscosity. In several cases soaking in a cryosolvent was required. Crystallographic data were collected with intense synchrotron radiation. Full data sets have been measured for native and derivatized crystals of 50S ribosomal subunits from H. marismortui as well as from their complexes with tRNA and nascent polypeptide chains, from the wild type and a mutant of 50S subunits from B. stearothermophilus, and from crystals of native and derivatized 30S ribosomal subunits from T. thermophilus.