gebhart/patrepeval huggingface dataset:
Pediatric Encephalopathy: Clinical, Biochemical and Cellular Insights into the Role of Gln52 of GNAO1 and GNAI1 for the Dominant Disease (2021)

Heterotrimeric G proteins are immediate transducers of G protein-coupled receptors-the biggest receptor family in metazoans-and play innumerate functions in health and disease. A set of de novo point mutations in <i>GNAO1</i> and <i>GNAI1</i>, the genes encoding the α-subunits (Gαo and Gαi1, respectively) of the heterotrimeric G proteins, have been described to cause pediatric encephalopathies represented by epileptic seizures, movement disorders, developmental delay, intellectual disability, and signs of neurodegeneration. Among such mutations, the Gln52Pro substitutions have been previously identified in <i>GNAO1</i> and <i>GNAI1</i>. Here, we describe the case of an infant with another mutation in the same site, Gln52Arg. The patient manifested epileptic and movement disorders and a developmental delay, at the onset of 1.5 weeks after birth. We have analyzed biochemical and cellular properties of the three types of dominant pathogenic mutants in the Gln52 position described so far: Gαo[Gln52Pro], Gαi1[Gln52Pro], and the novel Gαo[Gln52Arg]. At the biochemical level, the three mutant proteins are deficient in binding and hydrolyzing GTP, which is the fundamental function of the healthy G proteins. At the cellular level, the mutants are defective in the interaction with partner proteins recognizing either the GDP-loaded or the GTP-loaded forms of Gαo. Further, of the two intracellular sites of Gαo localization, plasma membrane and Golgi, the former is strongly reduced for the mutant proteins. We conclude that the point mutations at Gln52 inactivate the Gαo and Gαi1 proteins leading to aberrant intracellular localization and partner protein interactions. These features likely lie at the core of the molecular etiology of pediatric encephalopathies associated with the codon 52 mutations in <i>GNAO1</i>/<i>GNAI1</i>.
Identification of D motif epitopes in Staphylococcus aureus fibronectin-binding protein for the production of antibody inhibitors of fibronectin binding (1997)

A fibronectin-binding protein (FnBP) adhesin of Staphylococcus aureus possesses three 37- or 38-amino-acid motifs (D1, D2, and D3) that can each bind fibronectin (Fn) with low affinity and that in tandem comprise D1-3, a high-affinity Fn-binding domain. To identify epitopes for the generation of adhesion-blocking antibodies, rabbits were immunized with recombinant D1-3 or with a glutathione S-transferase fusion protein, GSTD1-3. Affinity-purified antibodies from the D1-3 immunization were poor inhibitors of Fn binding to S. aureus and recognized several different epitopes, with a preference for clusters of acidic amino acids that do not contribute to Fn binding. Antibodies generated with GSTD1-3 as an immunogen were more effective inhibitors, but concentrations in excess of 20 microg x ml-1 did not promote more than 50% inhibition. These antibodies were highly specific for amino acids 21 to 34 of D1 (D1(21-34)), which contain a sequence that is essential for Fn binding and are identical to D2 at 12 of 14 residues. Neither antibody preparation recognized D3(20-33) of the D3 motif, where the only homology to D1(21-34) and D2(21-34) comprises a sequence motif, GG(X3,4)(I/V)DF, that is critical to Fn binding. However, antibodies specific for both D1(21-34) and D3(20-33) could be obtained by using synthetic peptides corresponding to these sequences as immunogens. F(ab')2 fragments derived from these antibodies each caused 40 to 50% inhibition of Fn binding to S. aureus, and their ability to bind to purified FnBP was eliminated by competing Fn. However, mixtures of the two F(ab')2 preparations did not provide additive or synergistic inhibition of Fn binding. Therefore, inhibition of Fn binding to S. aureus requires antibodies specific for D1(21-34) and D3(20-33), but a mixture of antibodies specific for both sequences did not provide complete inhibition.
Biodegradable Poly(butylene succinate) Composites Reinforced by Cotton Fiber with Silane Coupling Agent (2013)

In this study, the use of cotton fiber (CF) as a filler in poly(butylene succinate) (PBS) and the effect of silane treatment on the mechanical properties, thermal stability, and biodegradability of PBS/CF composites are investigated. The results showed that the tensile strength of PBS was improved (15%–78%) with the incorporation of CF (10–40 wt%) and was further increased (25%–118%) when CF was treated with a silane coupling agent. Scanning electron microscopy (SEM) observation of the fracture surfaces of PBS/CF composites showed that there was slight improvement in fiber-matrix compatibility. Thermogravimetric (TG) analysis showed that the thermal stability of the composites was lower than that of neat PBS and decreased with increasing filler loading. The biobased carbon content of the composites increased with increasing CF content. The incorporation of CF (with and without silane treatment) in PBS significantly increased the biodegradation rate of the composites.
Properties of Nanocrystalline TiO2:V Thin Films as a Transparent Semiconducting Oxides (2009)

In this work the nanocrystalline TiO 2 thin films doped with vanadium in amount of 19 at.% and 23 at.% prepared by magnetron sputtering method have been presented. The transmission measurements shows that V -doped TiO 2 thin films were transparent in ca. 81% in the visible range of light spectrum. On the basis of electrical examinations it was found that fabricated TiO 2 :V thin films are semiconductors at room temperature and have different type of electrical conduction depending on the amount of vanadium dopant applied.
Thermal stability studies of fully silicided NiSi on Si-oxynitride and Hf-based high-κ gate stacks (2007)

The thermal stability of fully silicided (FUSI) NiSi∕Hf-based high-κ gate stacks upon exposure to a typical back-end-of-line (BEOL) N2 annealing or forming gas annealing is investigated. Our results demonstrate that there is no new phase formation during postsilicidation N2 annealing for blanket FUSI NiSi stack films. However, new phases are formed once the stack is exposed to forming gas annealing for 1h at 445°C. After a typical BEOL thermal budget annealing (350°C, 60min), Ni diffusion is observed into the Si channel through a thin 1.3nm Si-oxynitride layer, and increases with annealing temperature and time. The Ni penetration from the FUSI NiSi gate electrode structure is completely inhibited with thicker 5nm gate dielectric layers upon exposure to a similar BEOL annealing. The relatively weaker Ni–Ni bonds associated with Ni-rich phases coupled with grain boundary diffusion of liberated Ni likely contribute to Ni diffusion through dielectrics into the Si channel.
Immunological studies on mouse mammary tumors. III. Surface antigens reacting with tumor‐specific antibodies in immune adherence (1969)

Abstract Tumor‐specific antibodies reacted with an isografted ascitic form of mouse mammary tumor, MM2, with a higher sensitivity in an immune adherence test than in an immune cytotoxicity test. Immune adherence and immune adherence inhibition tests were applied for the detection of surface antigens of MM2 tumor reacting with tumor‐specific antibodies. After treatment with ether, ethanol, methanol, ethanol‐ether mixture, acetone, chloroform‐methanol mixture, deoxycholate, triton × 100, snake venoms, phospholipase A, or heating at 100° C for 15 minutes, the MM2 cells did not react with syngeneic antibodies in immune adherence. The reactivity of the cells was not suppressed by heating at 62° C for 15 minutes after treatment with deoxyribonuclease, ribonuclease, proteinases, neuraminidase, ultraviolet irradiation or freezing and thawing. In the immune adherence inhibition test, deoxycholate extract or triton × 100 extract of MM2 cells inhibited the immune adherence reactivity of syngeneic antibodies with MM2 cells, while the organic solvent extracts did not. This indicates that antigenic material was extractable in deoxycholate or triton × 100. After removal of surface antigens with 0.1% deoxycholate, fractions were prepared from treated MM2 cells. Fractions precipitated at 105,000 × g (microsomal fractions) as well as fractions precipitated at 11,000 × g (mitochondrial fractions) inhibited the immune adherence reactivity of tumor‐specific antibodies with MM2, indicating that antigens were also present in these fractions.
Perceptions of Curriculum Implementation Needs in Design and Technology (2004)

FGF10 promoted the development of the hSMGepiS/PC-derived salivary gland organoids by the expression of differentiation markers, structure formation, and response to neurotransmitters in vitro. Moreover, the hSMGepiS/PCs responded to the niche in mouse embryonic mesenchyme and further differentiated into salivary gland tissues with mature characteristics. Our study provides a foundation for the regenerative therapy of salivary gland diseases.
Extrusion of Wheat Gluten Plasticized with Glycerol: Influence of Process Conditions on Flow Behavior, Rheological Properties, and Molecular Size Distribution (1999)

ABSTRACT Gluten‐glycerol dough was extruded under a variety of processing conditions using a corotating self‐wiping twin‐screw extruder. Influence of feed rate, screw speed, and barrel temperature on processing parameters (die pressure, product temperature, residence time, specific energy) were examined. Use of flow modeling was successful for describing the evolution of the main flow parameters during processing. Rheological properties of extruded samples exhibited network‐like behavior and were characterized and modeled by Cole‐Cole distributions. Changes in molecular sizes of proteins during extrusion were measured by chromatography and appeared to be correlated to molecular size between network strands, as derived from the rheological properties of the materials obtained. Depending on operating conditions, extrudates presented very different surface aspects, ranging from very smooth‐surfaced extrudates with high swell to completely broken extrudates. The results indicated that extrudate breakup was caused by increasing network density, and some gliadins may have acted as cross‐linking agents. Increasing network density resulted in decreasing mobility of polymeric chains, and “protein melt” may no longer have been able to support the strain experienced during extrusion through the die. Increasing network density was reflected in increased plateau modulus and molecular size of protein aggregates. Increasing network structure appeared to be induced by the severity of the thermomechanical treatment, as indicated by specific mechanical energy input and maximum temperature reached.
Engineering erythrocytes as a novel carrier for the targeted delivery of the anticancer drug paclitaxel (2013)

Paclitaxel (PTX) is formulated in a mixture of Cremophor EL and dehydrated alcohol. The intravenous administration of this formula is associated with a risk of infection and hypersensitivity reactions. The presence of Cremophor EL as a pharmaceutical vehicle contributes to these effects. Therefore, in this study, we used human erythrocytes, instead of Cremophor, as a pharmaceutical vehicle. PTX was loaded into erythrocytes using the preswelling method. Analysis of the obtained data indicates that 148.8 μg of PTX was loaded/mL erythrocytes, with an entrapment efficiency of 46.36% and a cell recovery of 75.94%. Furthermore, we observed a significant increase in the mean cell volume values of the erythrocytes, whereas both the mean cell hemoglobin and the mean cell hemoglobin concentration decreased following the loading of PTX. The turbulence fragility index values for unloaded, sham-loaded and PTX-loaded erythrocytes were 3, 2, and 1 h, respectively. Additionally, the erythrocyte glutathione level decreased after PTX loading, whereas lipid peroxidation and protein oxidation increased. The release of PTX from loaded erythrocytes followed first-order kinetics, and about 81% of the loaded drug was released into the plasma after 48 h. The results of the present study revealed that PTX was loaded successfully into human erythrocytes with acceptable loading parameters and with some oxidative modification to the erythrocytes.
Application of Principal Component Analysis to Gene Expression Profiling by cDNA Microarray in Rice Seminal Root under Low pH Condition (2000)

イネの種子根において酸性条件で発現の変動する遺伝子のプロファイリング (profiling) を目的として, 1,265個のイネcDNAから構成されるcDNAマイクロアレイを使用した発現解析を行った. pH3.5の酸性処理した種子根と酸性処理しないものから抽出した全RNAを逆転写反応により蛍光標識して, イネcDNAマイクロアレイとのハイブリダイゼーションに供試した. 得られたシグナル強度データをもとに遺伝子発現プロファイルの比較を行った. シグナル強度は103から107の広い範囲に分布していたため, 分布の特性に基づき対数変換を行い, 反復間の比較から異常値を除外した後, 解析に供試した. 統計手法として主成分分析 (Principal Component Analysis: PCA) を適用し, 各種の処理間で発現の変動する遺伝子の判別を検討した結果, 第1主成分として処理と無関係な個々の遺伝子に固有のシグナル強度の差異が, 第2主成分として処理に依存したシグナル強度の変動がそれぞれ抽出された. 第2主成分の主成分スコアの上位2.5%に相当する遺伝子, すなわち, 酸性処理を加えた後にシグナル強度の増大が検出される遺伝子の中には, 植物細胞内pHの調節に関与すると推定されている複数の遺伝子が確認できた. 以上の結果から, PCAはcDNAマイクロアレイを利用した遺伝子発現解析に適した手法であると考えられた.
Determination of the bioavailability of gentamicin to the lungs following inhalation from two jet nebulizers (2005)

The MMAD and FPD data reflect the poor lung deposition of gentamicin identified by urinary excretion.
[Activation of the production of the tumor-necrosis factor by the combined action of lipopolysaccharide and muramyl dipeptide in vitro and in vivo]. (1987)

The effect of bacterial lipopolysaccharide (LPS), muramyl dipeptide (MDP) and their combination on the production of tumour necrosis factor by spleen cells in vitro and on tumour regression in vivo has been studied. TNF activity was detected in spleen cell supernatants and serum of mice treated with drugs, using L929 cells as targets. The combination of LPS and MDP was more effective in TNF production than each of the drugs used alone in vitro and in vivo. The injection of LPS and MDP to A/Sn mice with subcutaneous nodes of sarcoma SA-I resulted in total tumour necrosis. The treatment of mice with these drugs in water solutions was more effective, however, more toxic than the administration of LPS-treated splenocytes in MDP solution.
Complex dependence of substrate stiffness and serum concentration on cell‐force generation (2006)

Collagen is a widely used biomaterial in tissue engineering. Mechanical stimulation of cell-seeded collagen constructs and its effects on cell orientation, intracellular signaling, and molecular responses have been reported. Our aim was to study the transfer of applied mechanical load to resident cells in 3D collagen constructs. Stainless steel markers were embedded in constructs as reporters of micromovement and uniaxial (0-15%) strain was applied. Cell-seeded collagen constructs were also subjected to (0-15%) uniaxial strain and material responses recorded. The viscoelastic properties of collagen resulted in comparatively small movement of the marker bars relative to gel deformation. Cell seeding density of 1 million/mL had no significant effect on the viscoelastic properties of collagen for the range of strain tested. Our findings indicate that viscoelastic properties of collagen result in minimal force transfer of applied loads as recorded by movement of stainless steel markers. At higher strain rates as collagen got stiffer the movement decreased. These findings indicate that as cell-seeded collagen constructs mature in a bioreactor and become stiffer due to ECM production/deposition, mechanical stimulation will have to be tailored over time to account for increased stiffness of constructs in vitro to elicit predictable and consistent cellular responses.
A Handover Technique in a Dual-mode Base Station (2006)

핸드오버는 이동통신 단말기가 기지국과 기지국 사이를 이동해도 끊김없이 데이터 송수신이 가능하도록 해주는 기술이다 핸드오버 종류는 크게 두 가지로 구분되는데 네트워크 링크의 변경 없이 라디오 링크 연결만 변경되는 것을 수평(horizontal) 핸드오버라 하고, 서로 다른 네트워크 환경을 이동하는 것을 수직(vertical) 핸드오버로 정의할 수 있다. 본 논문은 SDR(Software Defined Radio)을 기반으로 W-CDMA망과 WiBro망을 동시에 지원하는 기지국내에서의 수직 핸드오프 프로토콜을 제안하고, 제안된 프로토콜을 상태천이도 및 페트리 넷을 이용하여 검증하고 또한 NS-2 시뮬레이터를 이용하여 제안된 프로토콜의 성능을 평가하였다 A handover is a technology that enables data transmission and receipt seamlessly while a mobile station moves from the current base station to another base station. The handover is basically classified into two types; a horizontal handover which changes a radio link only without changing a network link, and a vertical handover which changes both in heterogeneous networks. In this paper, we propose a vertical handover protocol in a dual base station which supports both of W-CDMA and WiBro networks based on SDR (Software Defined Radio), verify the rightness using a state transition diagram and a Petri-net, and evaluate the performance of the proposed protocol using NS-2 simulator.
A common disease-associated missense mutation in alpha-sarcoglycan fails to cause muscular dystrophy in mice (2008)

Limb-girdle muscular dystrophy type 2D (LGMD2D) is caused by autosomal recessive mutations in the alpha-sarcoglycan gene. An R77C substitution is the most prevalent cause of the disease, leading to disruption of the sarcoglycan-sarcospan complex. To model this common mutation, we generated knock-in mice with an H77C substitution in alpha-sarcoglycan. The floxed neomycin (Neo)-cassette retained at the targeted H77C alpha-sarcoglycan locus caused a loss of alpha-sarcoglycan expression, resulting in muscular dystrophy in homozygotes, whereas Cre-mediated deletion of the floxed Neo-cassette led to recovered H77C alpha-sarcoglycan expression. Contrary to expectations, mice homozygous for the H77C-encoding allele expressed both this mutant alpha-sarcoglycan and the other components of the sarcoglycan-sarcospan complex in striated muscle, and did not develop muscular dystrophy. Accordingly, conditional rescued expression of the H77C protein in striated muscle of the alpha-sarcoglycan-deficient mice prevented the disease. Adding to the case that the behavior of mutant alpha-sarcoglycan is different between humans and mice, mutant human R77C alpha-sarcoglycan restored the expression of the sarcoglycan-sarcospan complex when introduced by adenoviral vector into the skeletal muscle of previously created alpha-sarcoglycan null mice. These findings indicate that the alpha-sarcoglycan with the most frequent missense mutation in LGMD2D is correctly processed, is transported to the sarcolemma, and is fully functional in mouse muscle. Our study presents an unexpected difference in the behavior of a missense-mutated protein in mice versus human patients, and emphasizes the need to understand species-specific protein quality control systems.
Enzyme Immunoassay for a Characteristic Protein in the Animal Crude Drug Lumbricus. (1998)

A new method was developed to measure the content of a Lumbricus component in a traditional Chinese medicine (TCM). An antiserum specific to Lumbricus was elicited in a rabbit following immunization with a suspension of Lumbricus fragments. A characteristic antigen protein, 70 kDa, was found in Lumbricus and was purified almost to singleness using a column chromatography series of gel filtration and DEAE-Sepharose. A selected antibody enzyme immunoassay (SAEIA) was developed using the antiserum and the purified 70 kDa protein as a solid-phase antigen. The SAEIA was specific to Lumbricus species, and showed no cross-reaction with any crude drugs other than Lumbricus. This SAEIA detected 70 kDa protein in the amount of 10 ng/ml with excellent reproducibility (coefficient of variation=3.0%) and an EC50 of 0.24 microg/ml. Using this assay, Lumbricus levels were easily determined in a Lumbricus-based TCM Kazecoll, but not in the control Kazecoll (Kakkonto) prepared without Lumbricus. The SAEIA for 70 kDa protein was simple, accurate, reproducible and may provide a general analytical method for the quality control of Lumbricus-based TCMs.
Synthesis and properties of epitaxial thin films of c-axis oriented metastable four-layered hexagonal BaRuO3 (2000)

We have grown epitaxial thin films of c-axis oriented metastable four-layered hexagonal BaRuO3 on a (111) SrTiO3 substrate by 90° off-axis sputtering techniques. X-ray diffraction and transmission electron microscopy reveal that the films are single domains of c-axis four-layered hexagonal structures with an in-plane epitaxial arrangement of BaRuO3 [21̄1̄0]∥SrTiO3[110]. Surfaces with smooth terraces having a step height of a half unit cell (∼4.7 Å) have been observed by scanning tunneling microscopy. The in-plane electrical resistivity of the films is metallic, with a room temperature value of 810 μΩ cm and slightly curved temperature dependence. Their magnetic susceptibility is Pauli paramagnetic. The metastable layered metallic oxide can be used for understanding new solid-state phenomena and device applications.
P156 Differential characteristics of patients with inflammatory bowel disease onset in paediatric age compared with patients diagnosed in adulthood: Results from the CAROUSEL study of GETECCU (2018)

Population-based studies comparing the characteristics of paediatric IBD with IBD diagnosed in the adulthood are scarce. The aims of this study were (i) To compare the characteristics of IBD in patients diagnosed during childhood (≤16 years) with those from patients diagnosed in adulthood; (ii) to compare the incidence rate of initiation of immunosuppressants, biologic agents and surgery between both groups; and (iii) to evaluate the impact of the age at diagnosis on the initiation of immunosuppressants, biological agents and surgery. IBD patients from the ENEIDA registry—a large prospectively maintained Spanish database promoted by the Spanish Working Group in Crohn’s and Colitis (GETECCU), were included. In order to minimise the heterogeneity in the management of IBD patients, the inclusion was limited to those diagnosed after 1998, when the fist biological agent was approved for IBD in Europe. Patients diagnosed at ≤16 years comprised the paediatric cohort (PC), and those diagnosed >16 years were the adult cohort (AC). The cumulative incidences of immunosuppressive therapy, biological therapy and surgery, were estimated using Kaplan–Meier curves, which were compared by the log-rank test. Cox-regression analysis was performed to identify potential predictive factors to receive immunosuppressants, biological agents or surgery. From 35925 patients, there were 1630 paediatric cases (4.5%) and from them, 56 (3.4%) were very-early-onset IBD (<6 years). Median follow-up was 91 months in the PC and 72 months in the AC. The proportion of Crohn’s disease (CD), ileocolonic involvement and inflammatory behaviour at diagnosis was higher in the PC (Table 1). In the multivariate analysis, after adjusting for gender, type of IBD, extra intestinal manifestations, family history and smoking habit, the PC was associated with higher risk of immunomodulator use (hazard ratio [HR] = 1.47, 95% confidence interval [95% CI] = 1.38–1.58) and higher probability of receiving biologic treatment (HR = 1.53, 95% CI = 1.42–1.66). However, paediatric IBD was not associated with higher risk of surgery (Table 2). Paediatric IBD has differential characteristics in comparison with adult IBD. In addition, paediatric IBD seems to be associated with higher use of immunosuppressant and biologics during follow-up. Nevertheless, paediatric IBD is not associated with higher risk of surgery.
Identification of Insulin-Like Growth Factor-Binding Protein-3 (IGFBP-3) and IGFBP-2 in Human Follicular Fluid* (1990)

Two major insulin-like growth factor-binding proteins, IGFBP-2 and IGFBP-3, were identified by Western ligand blotting of human follicular fluid (FF) aspirated from luteinizing follicles during ovarian stimulation with human menopausal gonadotropin (LH and FSH). IGFBP-1 was also present in FF, and an IGFBP with a mol wt of 24000 daltons, present in serum, appeared to be absent in FF. Identification of IGFBP-1, IGFBP-2, and IGFBP-3 was confirmed using IGFBP-specific antisera. IGF-I and IGF-II levels were measured in patient serum and FF at the time of oocyte harvest, and serum levels of both peptides were consistently higher than FF levels. The finding of three major serum IGFBPs in human FF suggests that IGF regulation in the ovary is probably under complex control by this set of regulatory BPs.