gebhart/patrepeval huggingface dataset:
Src-related thrombocytopenia: a fine line between a megakaryocyte dysfunction and an immune-mediated disease (2022)

Src-related thrombocytopenia (SRC-RT) is a rare autosomal dominant, inherited platelet disorder resulting from the p.E527K heterozygous germline gain-of-function variant of Src. To date, genetic diagnosis of the disease has only been reported in 7 patients from 3 unrelated families. The clinical features ranged from isolated thrombocytopenia to complex syndromic manifestations characterized by thrombocytopenia, bleeding, myelofibrosis, splenomegaly, and bone disease. We report a new 3-generation kindred with the Src p.E527K variant. Patients presented with rather variable platelet counts (38-139 × 109/L), mildly impaired platelet function, >15% immature platelet fraction, and with a significant proportion of large-giant platelets. Four adults from the family were diagnosed with immune thrombocytopenia (ITP) and underwent splenectomy, achieving sustained platelet counts >75 × 109/L for several years; increases in platelet counts were also observed after corticosteroid therapy. Four of 7 Src p.E527K variant carriers showed immune defects and recurrent infections. In addition, a range of neurological symptoms, from specific language impairment to epilepsy, was seen in some family members. Patient platelets exhibited constitutive Src, Bruton tyrosine kinase, and phospholipase Cγ2 activation, and after stimulating CD19 cells by crosslinking surface immunoglobulin M, phosphorylated extracellular signal-regulated kinase (ERK) was significantly increased in B cells from individuals carrying the Src p.E527K substitution. In summary, in addition to causing impaired platelet production, SRC-RT may associate immune dysregulation and increased platelet consumption. In families in whom several members are responsive to ITP-directed therapies, an underlying Src p.E527K variant should be excluded.
Molecular Characterization of Clonotypic VDJ Sequences in Waldenstrom’s Macroglobulinemia Suggests Independent Transformation Events in the Development of Biclonal B Cells. (2006)

Abstract Malignant B lineage cells in Waldenstrom’s macroglobulinemia (WM) express a unique clonotypic VDJ associated with IgM. Studies of WM patients revealed a frequent incidence of biclonal B cells (16%) as defined by the presence of two distinct clonotypic VDJ sequences. This is the first report to estimate the frequency of biclonality in WM. Four WM cases are reported: two with distinct IgM clones (WM1-19 and WM1-09), one with distinct IgM/IgA clones (WM1-18), and one with related but diversified IgM/IgG clones (WM10). In 2 cases (WM1-19 and WM10), the two clonotypic signatures were found to be abundant in bone marrow (BM) but less frequent in blood, reminiscent of monoclonal WM cases. In WM1-19, single cell analysis showed that only one partner VDJ was expressed per cell, excluding the possibility of aberrant biallelic rearrangements. The distribution ratio between the two tumor clones was 2:1, suggestive of their mutual role in the clinical manifestation. In the other 2 cases (WM1-09 and WM1-18), partner clones were shown by CDR3 fragment analysis to be anatomically distinct, with one BM clonotypic signature and one in blood. In these 2 patients, the BM clone was hypermutated (6.2% and 3.8%) while the blood clone was germline or minimally mutated (0% and 1.0%). Partner clones lacked intraclonal diversity and Ig class switching, characteristic of malignant WM clones, suggesting relatively frequent transformation events throughout B lineage differentiation. The biological events leading to the appearance of clones in two different anatomic sites and the clinical implications remain to be understood. CDR3 fragment analysis in the longitudinal studies of WM1-09 and WM1-18 suggested that the repertoire of blood B cells may recover some level of diversity after successive cycles of treatment (WM1-18), while the persistence of the monoclonal peak (WM1-09) likely reflects a tumor that does not respond to treatment. In WM10, biclonal IgM/IgG B cell clones shared common VDJ gene rearrangement. The IgG clone displayed a higher mutation rate than did the IgM clone (9.7% vs. 6.1%). Increased somatic hypermutation in IgG consists mostly of replacement mutations that are clustered within the CDR regions, strongly supporting a contention that the IgG clone has undergone affinity maturation. There are 11 point mutations in the IgM that are not present in the IgG, suggesting that the two clones are distinctly different and belong to different branches of the genealogical tree. These distinct mutational signatures suggest that biclonal IgM/IgG clones are unlikely derived from clonal evolution. Overall, our results suggest that for the four biclonal WM evaluated here, the partner B cell clones appear to have undergone separate transformation events in the development of biclonality. The extent to which each partner clone contributes to disease progression and death, whether separately or in synergy, is as yet unknown.
High-speed graphene based quantum-optical interconnect design (2015)

While traditional transistor scaling in integrated circuit industry is going on one side, interconnect scaling has become the performance-limiting factor for new designs on the other side. The increasing influence of interconnect parasites on crosstalk noise and RLC delay as well as electro-migration and power dissipation concerns have stimulated the introduction of low-resistivity copper and low-permittivity (k) dielectrics to provide performance and reliability enhancement. Additionally, entirely new technology such as optical interconnect is required to address future global routing needs and sustain performance improvement.
Measurement of Turbulence Intensity in the Center of the Canine Ascending Aorta With a Hot-Film Anemometer (1983)

The blood flow velocity near the central axis of the canine ascending aorta was measured with a hot-film anemometer. The cardiac output and the heart rate were controlled at will by means of an extracorporeal circulation and by atrial pacing. The turbulent component of the blood flow velocity was calculated using an ensemble average technique. Ensemble average turbulent intensity was also calculated to show the time course of turbulence in the aorta. The ratio of the mean turbulence intensity to the time mean sectional average velocity in the aorta was constant in most animals regardless of the changes in fluid mechanical parameters. The correlation between the frequency parameter and the relative mean turbulence intensity was weakly positive. The power spectrum of the turbulence was also calculated.
Circuits électroniques neuro-inspirés : vers une consommation énergétique ultra optimisée ? (2016)

National audience
2014 Twelfth annual conference on privacy, security and trust (2014)

International audience
Basic Components and Fiber Optic Passive Components: Status and Trends in Japan (1986)

This paper reviews the status and trends of Japanese activities in fiber optic passive components. It covers the fiber manufacturing techniques (VAD method), several special fibers such as polarization-holding fibers, dispersion-shifted fibers, infrared fibers, metal coated fibers, and hydrogen-induced loss problems, as well as various kinds of fiber cables such as trunk communication cable, subscriber cable, submarine cable, and optical fiber composite overhead ground wire. The paper also discusses the problems of splicing, connecting, and coupling with respect to future trends and provides information on progress in Japan in the expanding field of communication technique.
Cyclin D3 Is Required for the Germinal Center Reaction (2008)

Abstract Germinal Centers (GC) of secondary lymphoid tissues are critical to mounting a high-affinity humoral immune response. B cells within the GC undergo rapid clonal expansion and selection while diversifying their antibody genes through class switch recombination and somatic hypermutation. Although it is generally believed that GC B cells employ a unique proliferative program to accommodate these processes, very little is known about how the GC-associated cell cycle is orchestrated. The D-type cyclins are important regulators of the G1 phase of the cell cycle and are the ultimate targets of many mitogenic and oncogenic stimuli. The Cyclin D3 gene is rearranged and over-expressed in certain mature B cell malignancies, and its overexpression has been reported to predict poor clinical outcome in patients with diffuse large B cell lymphoma. It has been observed that during their development, B cells switch from expressing cyclin D2 to cyclin D3 when they are recruited into the GC response. It is unclear, however, whether this switch simply reflects a change in the transcription factors that govern cyclin expression or serves a biological mandate. Here we report that mice deficient in cyclin D3 are profoundly impaired in their ability to form GCs as measured by immunohistochemistry and flow cytometry. Production of antigen-specific antibodies and affinity maturation, as ascertained by ELISA, are concomitantly reduced in these animals. These phenotypes can be at least partially explained by a significant block in the G1-phase of the cell cycle of GC B cells in vivo. Interestingly, this block in the G1-S transition is observed despite an apparent compensatory increase in cyclin D2 expression. In addition, naive B cells activated in vitro by either LPS or LPS and IL-4 display only minor changes in cell-cycle profile, suggesting that a specific requirement for cyclin D3 is unique to GC B cells. We also find moderately reduced Bcl6 mRNA expression in both naïve and GC B cells from the cyclin D3 knockout mice. Since Bcl6 is a master regulator of the GC response, decreased activity of this transcriptional repressor may further contribute to the severity of the GC phenotype. This is the first demonstration that cyclin D3 plays a unique role during the GC response in that it is required for its optimal structure and function. In addition to expanding appreciation for the cell type- and tissue-specific functions of the three D-type cyclin molecules, our findings have implications for understanding the role of Cyclin D3 in human B cell lymphomas.
Hydrogen Ingress into Copper-Nickel Alloys (1994)

Hydrogen (H) ingress into two copper (Cu)-nickel (Ni) alloys — a commercial 77% Cu-15% Ni alloy (aged) and alloy K-500 (UNS N05500, aged and unaged) — was studied using a technique referred to as hydrogen ingress analysis by potentiostatic pulsing (HIAPP). Anodic current transients obtained for these alloys in an acetate buffer (1 mol L–1 acetic acid + 1 mol L–1 sodium acetate [NaAc]) were analyzed using a diffusion-trapping model to determine trapping constants and H entry fluxes. A small increase was observed in the irreversible trapping constant for alloy K-500 with aging. Trapping constants of the aged alloys were similar within the limits of uncertainty, but H entry flux for the 77% Cu alloy was lower than that for aged or unaged alloy K-500. The lower flux may have accounted, at least partly, for the Cu alloy’s higher resistance to H embrittlement. Trap densities were consistent qualitatively with levels of sulfur (S) and phosphorus (P) in the two alloys. This finding supported an assumption that S and P provided the primary irreversible traps.
On-chip debug support for embedded Systems-on-Chip (2003)

This paper presents an on-chip hardware architecture to support application software development for embedded Systems-on-Chips (SoC). This architecture provides debug support for one manufacturer's complete SoC platform. This includes a significant number of 16-bit and 32-bit microcontroller and DSP cores, spanning a multitude of application specific systems ranging from wireless systems to engine controllers. The debug support architecture is modular and can be divided into three main components: i) processor-specific debug resources, ii) a serial communication interface to connect the SoC with a debug host computer system and iii) a number of interconnection links to communicate between the serial communication interface and the processor debug resources. This modular approach allows the debug support architecture to be adapted to almost any system configuration (including multiple processor cores), while keeping the impact on silicon real estate for production devices at a minimum.
Packet leashes: a defense against wormhole attacks in wireless networks (2004)

As mobile ad hoc network applications are deployed, security emerges as a central requirement. In this paper, we introduce the wormhole attack, a severe attack in ad hoc networks that is particularly challenging to defend against. The wormhole attack is possible even if the attacker has not compromised any hosts, and even if all communication provides authenticity and confidentiality. In the wormhole attack, an attacker records packets (or bits) at one location in the network, tunnels them (possibly selectively) to another location, and retransmits them there into the network. The wormhole attack can form a serious threat in wireless networks, especially against many ad hoc network routing protocols and location-based wireless security systems. For example, most existing ad hoc network routing protocols, without some mechanism to defend against the wormhole attack, would be unable to find routes longer than one or two hops, severely disrupting communication. We present a new, general mechanism, called packet leashes, for detecting and thus defending against wormhole attacks, and we present a specific protocol, called TIK, that implements leashes.
Transport and charge detection spectroscopy of few electron quantum dot molecular states. (2005)

Few electron lateral quantum dot molecules have been fabricated and studied using both transport and charge detection techniques. Such devices are ultimately planned for quantum information applications such as charge and spin based qubits. Measurements have been performed for several specific configurations (n,m) relevant for quantum information where n and m refer to the measured number of electrons in each dot e.g. (5,5) the lowest occupancy configuration with a filling factor 2 state. Measurements were made as a function of magnetic field and the tunnel coupling between the dots.
Learning Feedforward Control of a Hydraulic Clutch Actuation Path Based on Policy Gradients (2020)

The hydraulic clutch actuation path used in heavy duty transmissions often shows a lot of variability due to manufacturing tolerances and ageing effects. Reason for this are in particular varying friction coefficients in the spools and external factors such as compliance with the specified service intervals or the choice of hydraulic fluid. As a direct consequence, the shift quality typically varies from one transmission to the next. To resolve this problem, this paper presents a machine learning algorithm for the feedforward control of the hydraulic clutch actuation path, a model-based and a data-based feedforward approach. The two approaches are evaluated and compared with each other in simulations with a high-fidelity model. As it turns out, the model-based version is to be preferred and therefore used in a real world evaluation on an embedded controller and a 16 tons wheel loader.
INDOT Guidance Document for In-Situ Soil Flushing (2007)

Many of the Indiana Department of Transportation (INDOT) sites are contaminated with aromatic hydrocarbons and chlorinated solvents; these contaminants often occur in the form of light non-aqueous phase liquids (LNAPLs) or dense non-aqueous phase liquids (DNAPLs). Considerable effort has recently been focused on developing in-situ technologies for removing or destroying NAPL source zones, and several potentially viable methods have emerged including cosolvent-enhanced source zone removal. Most recently, site-specific remedial designs involving a combination of in-situ methods (often referred to as a treatment train) have been proposed for which data are still sparse. The primary goal of the laboratory experiments is to assess the utility and effectiveness of a biofriendly cosolvent ethyl lactate for source zone flushing through enhanced dissolution mechanisms followed by assessing enhanced biological removal of contaminant residuals by cosolvent residuals. Ethyl lactate residuals after source zone remediation can serve as a substrate for indigenous microorganisms to induce anaerobic conditions and enhance in-situ reductive dehalogenation, but whether or not this process would further facilitate complete mineralization or if it too would stall at undesirable metabolites is unknown. Results from these lab evaluations involving batch tests and one-dimensional (1-D) and two-dimensional (2-D) flow systems will contribute to assessing the value of this treatment train approach for relevant INDOT sites and to developing an effective strategy for using this approach under field conditions. Ethyl lactate was found to be very efficient in recovering DNAPL mass in source zone. The residual amount of EL not only poses no harm to indigenous microbial population in a DNAPL contaminated site, but also serves as electron donor to promote the microbial dehalogenation of chlorinated contaminants, such as perchloroethene (PCE) and trichloroethylene (TCE) in the down gradient plume. Therefore, the flushing agent, EL, can be left behind at low concentrations to facilitate biodegradation in the plume. The soils used were from INDOT sites selected based on input from INDOT staff regarding amenability to future site remediation via the proposed treatment train. Site selection and assessment required travel by senior personnel to evaluate the nature and extent of contamination and by research assistants to collect soil samples for laboratory experiments.
Construction of Coassociative submanifolds in $\mathbb{R}^{7}$ and $\Lambda^{2}_{-} S^{4}$ with symmetries (2013)

We construct explicit examples of coassociative submanifolds in $\mathbb{R}^{7}$ and $\Lambda^{2}_{-} S^{4}$ invariant under the action of ${\rm SU}(2)$ and $T^{2} \times \mathbb{R}_{>0}$ by the symmetries method.
Influenza Antigens NP and M2 Confer Cross Protection to BALB/c Mice against Lethal Challenge with H1N1, Pandemic H1N1 or H5N1 Influenza A Viruses (2021)

Influenza hemagglutinin (HA) is considered a major protective antigen of seasonal influenza vaccine but antigenic drift of HA necessitates annual immunizations using new circulating HA versions. Low variation found within conserved non-HA influenza virus (INFV) antigens may maintain protection with less frequent immunizations. Conserved antigens of influenza A virus (INFV A) that can generate cross protection against multiple INFV strains were evaluated in BALB/c mice using modified Vaccinia virus Ankara (MVA)-vectored vaccines that expressed INFV A antigens hemagglutinin (HA), matrix protein 1 (M1), nucleoprotein (NP), matrix protein 2 (M2), repeats of the external portion of M2 (M2e) or as tandem repeats (METR), and M2e with transmembrane region and cytoplasmic loop (M2eTML). Protection by combinations of non-HA antigens was equivalent to that of subtype-matched HA. Combinations of NP and forms of M2e generated serum antibody responses and protected mice against lethal INFV A challenge using PR8, pandemic H1N1 A/Mexico/4108/2009 (pH1N1) or H5N1 A/Vietnam/1203/2004 (H5N1) viruses, as demonstrated by reduced lung viral burden and protection against weight loss. The highest levels of protection were obtained with NP and M2e antigens delivered as MVA inserts, resulting in broadly protective immunity in mice and enhancement of previous natural immunity to INFV A.
SOLID HOUSEHOLD WASTE DISPOSAL BY DOUBLE STAGE BURNING ON WALKING BEAM AND IN THE BUBBLED MELT (2021)

State of the problem of solid waste disposal at landfills has been analyzed. A combined method of two stages of solid waste incineration during the heating season has been proposed. The results of calculations of thermal balances of evaporation processes, burning on a movable bottom and in a bubbled melt, heating of air in the recuperator, heating of water in the contact heat exchanger, boiler and in the hot water supply system has been presented. It is shown that the elimination of the source of pollution in the form of solid waste in a two-stage method of incineration: on a walking step and then in a bubbled melt — makes it possible to get rid of secondary solid waste generated by traditional incineration. As a result of melting in the bubbled melt gases are formed, which contain the products of combustion and decomposition of solid waste and slag consisting of silicates and metal oxides. Bibl. 10, Fig. 3, Table 1.
c-erbB-2 amplification in node-negative human breast cancer. (1989)

c-erbB-2 gene analysis by Southern and DNA dot blot methods was done in 66 tumor samples from patients with histologically node-negative breast cancer. The c-erbB-2 gene was amplified 2- to greater than 8-fold in 13 tumors (20%). None of 59 tumors that were examined by the Southern method showed c-erbB-2 gene rearrangement. c-erbB-2 amplification was analyzed in relation to other prognostic factors. The c-erbB-2 gene was amplified in five of 36 (14%) diploid and eight of 30 (27%) aneuploid tumors. Thirteen of 54 (24%) tumors with nuclear Grade 1 or 2 displayed c-erbB-2 amplification, whereas none of 12 tumors with nuclear Grade 3 did. No correlation was observed with estrogen receptor content, tumor size, histological type, or age of patients. The median follow-up date for these patients was 85+ mo. Of 13 patients whose tumors showed c-erbB-2 amplification, six patients (46%) developed recurrence, and five patients (38%) died of metastatic disease. In contrast, of 53 patients whose tumors did not show c-erbB-2 amplification, 15 patients (28%) developed recurrence, and seven patients (13%) died of disease. In conclusion, our results show that c-erbB-2 gene amplification was more frequent in aneuploid tumors and tumors with poor nuclear grade. c-erbB-2 amplification may be considered a possible prognostic factor in node-negative breast cancer.