bluuebunny/crossref_metadata_2025_split huggingface dataset:
GPVI down-regulation in murine platelets through metalloproteinase-dependent shedding
Authors: Strehl Amrei , Piffath Crystal , Prostredna Miroslava , Wagner Denisa , Bergmeier Wolfgang , Rabie Tamer , Nieswandt Bernhard 
Published: April 2004

SummaryPlatelet interaction with subendothelial collagen is crucial for hemostasis and thrombosis. Under conditions of elevated shear, platelet adhesion and activation on collagen requires the coordinated action of glycoprotein (GP) Ib and GPVI, which may be physically and functionally linked in the platelet membrane. While the surface expression of GPIb can be down-regulated by internalization and/or proteolytic cleavage of a 130 kDa fragment of GPIbα (glycocalicin, GC), very little is known about the cellular regulation of GPVI. We have recently shown that GPIb on platelets is cleaved by metalloproteinase-dependent mechanisms in response to mitochondrial injury. In the current study, we examined a possible role of platelet metalloproteinases in the regulation of GPVI. Mitochondrial injury induced by incubation of mouse platelets with carbonyl cyanide m-chlorophenyl-hydrazone (CCCP) severely affected the cells´ responses to collagen or the GPVI-specific agonist, collagen related peptide (CRP), but not to thrombin or the stable thromboxane A2 analog U46619.This defect was due to a rapid proteolytic cleavage of GPVI, as shown by the release of the 55 kDa extracellular domain into the supernatant. Both the proteolysis of GPVI and the loss of its activity were inhibited in the presence of the broad range metalloproteinase inhibitor, GM6001. Platelet stimulation with thrombin or CRP, however, resulted in marked metalloproteinase-dependent shedding of GPIbα, but not GPVI suggesting that different metalloproteinases are involved in the regulation of the two receptors or, alternatively, an additional signal is required to render GPVI susceptible to cleavage.
Low density lipoprotein receptor-related protein: regulation of the plasma membrane proteome
Authors: Wu Lihua , Salicioni Ana , Gonias Steven 
Published: April 2004

SummaryProteins in the plasma membrane anchor the cell within its microenvironment and sense changes occurring outside the cell. The anchoring interactions are cell type-specific and may involve adjacent cells or extracellular matrix proteins (ECMPs). In development, wound healing, and in various forms of pathology, including thrombosis and atherosclerosis, the microenvironment of the cell may change rapidly and dramatically. How the cell responds is strongly dependent on the protein composition of its plasma membrane, which we refer to as the plasma membrane proteome. Processes that regulate the plasma membrane proteome may alter cellular response. Low density lipoprotein receptor-related protein-1 (LRP-1) is a member of the LDL receptor family; however, LRP-1 and other less well studied members of this gene family demonstrate multiple activities unrelated to lipid homeostasis. LRP-1 binds and internalizes numerous, structurally diverse ligands, delivering most but not all these ligands to lysosomes for degradation. The intracellular tail of LRP-1 binds signaling adaptor proteins and thus may function in cell signaling. Biological activities of LRP-1 include antigen presentation, phagocytosis, removal of apoptotic cells, and regulation of vascular permeability. This review focuses on an emerging view of LRP-1 activity, in which LRP-1 regulates the protein composition of the plasma membrane and thereby “models” or “landscapes” the cell surface. In some cases, plasma membrane modeling results from the binding to bifunctional ligands or intracellular adaptor proteins, so that LRP-1 is bridged to another plasma membrane protein and the entire complex undergoes endocytosis. Membrane proteins already known to be subject to this form of regulation include urokinase-type plasminogen activator receptor, amyloid precursor protein, tissue factor, and αV-containing integrins. LRP-1 also controls the plasma membrane proteome by regulating maturation and transport of proteins in the secretory pathway. At the same time, LRP-1 serves as a receptor for specific ECMPs, including fibronectin and thrombospondin. Although ECMP-binding to LRP-1 results in endocytosis and catabolism, these receptor-ligation events also may be coupled, directly or indirectly, to cell-signaling. Based on these novel activities, LRP1 emerges as a protein capable of modeling the interface of the cell with its microenvironment.
Validation of a risk score identifying patients with acute pulmonary embolism, who are at low risk of clinical adverse outcome
Authors: Bandelier Patrick , Aujesky Drahomir , Cornuz Jacques , Roy Pierre-Marie , Bounameaux Henri , Perrier Arnaud , Nendaz Mathieu 
Published: April 2004

SummaryA clinical predictive model that accurately identifies patients with pulmonary embolism who are at low risk of adverse medical outcomes may be useful for management decisions, such as outpatient treatment. We aimed to externally validate a previously derived prognostic score identifying emergency ward patients with acute pulmonary embolism at low risk of 3- month complications. One hundred and ninety-nine consecutive patients with proven pulmonary embolism were included from the emergency centres of three teaching and general hospitals. Adverse outcomes, such as death, major bleed, or recurrent venous thromboembolism, were recorded during a 3-month follow-up. We retrospectively computed the prognostic score for each patient and determined its predictive accuracy at different threshold values. The overall 3-month risk of adverse event after the diagnosis of pulmonary embolism was 9.5%. At a threshold of 2 points, eight patients with scores at or below the cut-off (5%; 95% CI 2.6–9.6) and 11 patients with scores above this cut-off (27.5%; 95% CI 16.1–42.8) presented a complication. The negative predictive value for an adverse outcome was 95.0% (95% CI 90.4–97.4). The receiver operating characteristic curve derived from the score distribution had an area of 0.77 (95% CI 0.65–0.89). This compared favourably with the characteristics of the derivation study. We conclude that the Geneva risk score has an acceptable predictive accuracy to identify patients with pulmonary embolism at low risk for 3-month adverse outcomes. Whether this score remains accurate and useful in clinical practice should be determined in a prospective multicentre validation study.
Recombinant expression of mutations causing von Willebrand disease type Normandy: characterization of a combined defect of factor VIII binding and multimerization
Authors: Lenk Harald , Obser Tobias , Oldenburg Johannes , Oyen Florian , Schneppenheim Sonja , Schwaab Rainer , Will Kerstin , Budde Ulrich , Schneppenheim Reinhard 
Published: April 2004

SummaryVon Willebrand disease type Normandy (VWD 2N) is caused by mutations at the factor VIII (FVIII) binding site of VWF, located at the amino-terminus of mature VWF. It is inherited in a recessive fashion and both homozygous and compound heterozygous mutations have been identified. Homozygous mutations are correlated with a clinical phenotype indistinguishable from mild hemophilia A by conventional laboratory tests, whereas compound heterozygosity with a quantitative defect may appear as VWD type 1 (VWD1). We have now identified and expressed a novel heterozygous mutation (Y795C) which is responsible for both, a defective FVIII-binding and aberrant multimers in a female patient with mild FVIII deficiency. Additionally we expressed another mutation (E787K), previously identified by us in a male patient with a severe ‘pseudohemophilic’ phenotype. Analysis of the FVIII binding and the multimer structure of the respective recombinant VWF mutants reproduced the observed phenotype: the FVIII binding defect in addition to the aberrant multimer structure of the patient with Y795C and the FVIII binding defect only, in the patient with E787K. Our results demonstrate the causative nature of the two mutations and emphasize the impact of ‘cysteine mutations’ on the multimer structure of VWF.
Reduced-intensity allogeneic hematopoietic stem cell transplantation for myelodysplastic syndrome and acute myeloid leukemia with multilineage dysplasia using fludarabine, busulphan, and alemtuzumab (FBC) conditioning
Authors: Ho Aloysius Y. L. , Pagliuca Antonio , Kenyon Michelle , Parker Jane E. , Mijovic Aleksandar , Devereux Stephen , Mufti Ghulam J. 
Published: April 2004

Abstract
Reduced-intensity conditioned (RIC) hematopoietic stem cell transplantation (HSCT) has improved the accessibility of transplantation in patients previously ineligible. We report the results of allografting following conditioning with fludarabine, busulphan, and alemtuzumab in 62 patients with myelodysplastic syndromes (MDSs) (matched sibling donors [24] or volunteer unrelated donors [VUDs, 38]). The median age for sibling recipients was 56 years (range, 41-70 years) and for VUD recipients, 52 years (range, 22-65 years), with a median follow-up (survivors) of 524 days (range, 93-1392 days) and 420 days (range, 53-1495 days), respectively. The nonrelapse mortality (NRM) at days 100, 200, and 360 was 0%, 5%, and 5%, respectively, for siblings and 11%, 17%, and 21%, respectively, for VUD. The overall survival at one year was 73% for siblings and 71% for VUDs, with a disease-free survival (DFS) of 61% and 59%, respectively. The prognostic significance of the International Prognostic Scoring System (IPSS) was preserved. Of recipients, 86% achieved full-donor chimerism. The cumulative incidence at day 100 of grades III to IV graft-versus-host disease (GVHD) for VUD recipients was 9% and for sibling recipients, 0%. There were 26 patients (16 sibling and 10 VUD) who received donor lymphocyte infusion (DLI) at a median of 273 days (range, 126-1323 days). RIC allogeneic HSCT using this protocol appears to be safe and permits durable donor engraftment. Longer follow-up is required to confirm any potential survival advantage. (Blood. 2004;104:1616-1623)
Declining stroke rates in Californian children with sickle cell disease
Authors: Fullerton Heather J. , Adams Robert J. , Zhao Shoujun , Johnston S. Claiborne 
Published: April 2004

Abstract
Although the Stroke Prevention Trial in Sickle Cell Anemia (STOP) demonstrated the efficacy of blood transfusions for primary stroke prevention in high-risk children with sickle cell disease (SCD) in 1998, the impact of this trial on public health has not been studied. Our objective was to determine whether stroke rates in Californian children with SCD have declined since 1998. Using a California-wide hospital discharge database, we identified all first admissions for stroke in children with SCD from 1991 through 2000. Annual stroke incidence rates were calculated as the number of admissions divided by the estimated population of Californian children with SCD in that year. For 1991-2000, 93 children with SCD were admitted to Californian hospitals with a first stroke during 12 030 person-years of follow-up; 92.5% were ischemic and 7.5% hemorrhagic. Overall, the rate of first stroke was 0.77/100 person-years. For the study years 1991-1998, the rate for first stroke was 0.88/100 person-years compared to 0.50 in 1999 and 0.17 in 2000 (P &lt; .005 for trend). Since the publication of the STOP study in 1998, annual rates of admissions for first stroke for Californian children with SCD have declined. (Blood. 2004;104:336-339)
Diabetes Mellitus and Optic Atrophy: A Study of Wolfram Syndrome in the Lebanese Population
Authors: Medlej R. , Wasson J. , Baz P. , Azar S. , Salti I. , Loiselet J. , Permutt A. , Halaby G. 
Published: April 2004

Abstract
Wolfram syndrome (WFS) is a rare hereditary neurodegenerative disorder also known as DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness). WFS seems to be a heterogeneous disease that has not yet been fully characterized in terms of clinical features and pathophysiological mechanisms because the number of patients in most series was small.
In this study we describe 31 Lebanese WFS patients belonging to 17 families; this, to our knowledge, is the largest number of patients reported in one series so far. Criteria for diagnosis of WFS were the presence of insulin-dependent diabetes mellitus and optic atrophy unexplained by any other disease. Central diabetes insipidus was found in 87% of the patients, and sensorineural deafness confirmed by audiograms was present in 64.5%. Other less frequent features included neurological and psychiatric abnormalities, urodynamic abnormalities, limited joint motility, cardiovascular and gastrointestinal autonomic neuropathy, hypergonadotropic hypogonadism in males, and diabetic microvascular disease. New features, not reported in previous descriptions, such as heart malformations and anterior pituitary dysfunction, were recognized in some of the patients and participated in the morbidity and mortality of the disease. Genetic analysis revealed WFS1 gene mutations in three families (23.5%), whereas no abnormalities were detected in mitochondrial DNA.
In conclusion, WFS is a devastating disease for the patients and their families. More information about WFS will lead to a better understanding of this disease and hopefully to improvement in means of its prevention and treatment.
Idiopathic Central Diabetes Insipidus Is Associated with Abnormal Blood Supply to the Posterior Pituitary Gland Caused by Vascular Impairment of the Inferior Hypophyseal Artery System
Authors: Maghnie Mohamad , Altobelli Monica , di Iorgi Natascia , Genovese Eugenio , Meloni Giulia , Manca-Bitti Maria Luisa , Cohen Amnon , Bernasconi Sergio 
Published: April 2004

Abstract
Central diabetes insipidus (CDI) has been linked to vascular central nervous system damage, although the pathophysiology of the mechanism has never been perfectly understood. Indeed, the vascular system of human pituitary gland has rarely been the subject of rigorous investigation except at postmortem. Recently, studies of pituitary gland blood supply have been carried out by means of a time evaluation of pituitary gland enhancement with noninvasive dynamic magnetic resonance (MR) imaging after contrast medium injection. In the present study, we decided to investigate the status of posterior pituitary blood supply by evaluating vascular pituitary patterns in a group of 19 patients with idiopathic CDI in whom previous standard MR imaging had failed to identify causal specific lesions. The control group was composed of 55 subjects with a median age of 12 yr (range, 4.2–17 yr) who had idiopathic isolated GH deficiency and normal pituitary morphology and 15 young adults (18–25 yr) who had normal pituitary gland and no endocrine dysfunction.
Nineteen patients (12 females and seven males), ranging in age at the time of diagnosis of CDI from 0.5–14.9 yr (median, 5 yr), were examined with dynamic MR imaging between 1990 and 1997 at a median age of 14.1 yr (range, 5.0–26.3 yr). CDI was diagnosed according to clinical findings of polyuria and polydipsia, water deprivation test, and desmopressin acetate therapeutic trial. All of the patients had permanent CDI and were being treated with satisfactory results with desmopressin, two to three times daily, either intranasally or orally. The previous MR imaging findings of the 19 CDI patients had shown the absence of posterior pituitary hyperintensity, normal pituitary stalk, and normal anterior pituitary size.
Enhancement of the straight sinus, representing a temporal reference point and occurring in normal subjects simultaneously to that of the posterior pituitary gland, was observed in all subjects after iv gadopentetate dimeglumine administration, with no substantial differences between patients and controls. However, the enhancement of the posterior pituitary lobe occurred simultaneously with the enhancement of the straight sinus in all of the controls but in only 14 of the 19 patients with CDI. In the remaining five patients, the enhancement of the straight sinus was not associated with the expected contrast enhancement of the posterior pituitary gland, suggesting abnormal blood supply to the posterior pituitary lobe. This is in keeping with vascular impairment of the inferior hypophyseal artery system and suggests that abnormal blood supply to the posterior pituitary gland is associated with what, until now, has been considered idiopathic CDI.
Molecular Orientation Control in Amphiphilic α-Helical Copolypeptide Monolayer at Air/Water Interface
Authors: Yokoi Hidenori , Kinoshita Takatoshi 
Published: April 2004

Abstract
Molecular orientation of amphiphilic α-helical di-block type copolypeptide PLLysZ25–P(MLG42/LGA18) was controlled in a monolayer at an air/water interface by controlling the secondary structure of hydrophilic segment of the peptide. The orientation of the peptide was investigated by surface-area (π-A) measurement, FT-IR reflection-absorption spectroscopy (RAS), and atomic force microscopy (AFM).
Microwave-Assisted Solvothermal Synthesis of Radial ZnS Nanoribbons
Authors: Liu Xiaoying , Tian Bozhi , Yu Chengzhong , Tu Bo , Zhao Dongyuan 
Published: April 2004

Abstract
Using microwave as the heating method, radial ZnS nanoribbons have been successfully synthesized for the first time via a solvothermal procedure in enthylenediamine, combined with thermal treatment in a nitrogen flow. The resulted nanoribbons have a length up to 700 nm, a width of 10–20 nm, and a typical thickness of 3 nm.
Highly Porous Organic Nanoparticles Formed from Supercritical Carbon Dioxide Mediated Sol–Emulsion–Gel Method
Authors: Lee Jun-Young , Kim Jung-Hyun 
Published: April 2004

Abstract
Highly porous organic nanoparticles have been prepared by adapting a novel method, sol–emulsion–gel process, through stable dispersion of nanometer-scaled emulsion droplets into continuous phase of supercritical carbon dioxide (ScCO2), sol–gel chemistry in emulsion droplets, and then supercritical drying. This principle offers faithful preparation of nanoparticles (35–90 nm in diameter) having pores of 1–3 nm in radius and high specific surface area (over 2000 m2/g).
High Birefringent Bisdiynes and Hexatriynes Based on Double Elimination of β-Substituted Sulfones
Authors: Ye Fangguo , Orita Akihiro , Yaruva Jayamma , Hamada Tatsuya , Otera Junzo 
Published: April 2004

Abstract
In order to realize high birefringence, acetylenes with long molecular size such as bis(phenyldiynyl)benzenes 1 and diphenylhexatriynes 4 are synthesized successfully on the basis of double elimination of the β-substituted sulfones which can be prepared easily by reaction of benzyl or propargyl sulfones with propynal derivatives.
Inclusion Formation between 1D Coordination Polymer Host and CS2 through Vapor Adsorption
Authors: Takamizawa Satoshi , Nakata Ei-ichi , Saito Teruo 
Published: April 2004

Abstract
The formation of CS2 inclusion crystals through vapor adsorption was characterized to show that large CS2 guests are tightly confined in the channel supported on the π orbital of the conjugate planes of the channel surface: the manner of guest alignment is strongly influenced by the structures of the guest and channel.
A Novel Mn12 Single-molecule Magnet with a μ3-Methanesulfonate Bridge
Authors: Kuroda-Sowa Takayoshi , Handa Tetsuji , Kotera Takehiro , Maekawa Masahiko , Munakata Megumu , Miyasaka Hitoshi , Yamashita Masahiro 
Published: April 2004

Abstract
The first Mn12 complex with μ3-methanesulfonate bridge was observed and its single-molecular magnetic property was characterised.
Highly Ordered Mesoporous Ni Particles Prepared by Electroless Deposition from Lyotropic Liquid Crystals
Authors: Yamauchi Yusuke , Yokoshima Tokihiko , Mukaibo Hitomi , Tezuka Masato , Shigeno Tetsuro , Momma Toshiyuki , Osaka Tetsuya , Kuroda Kazuyuki 
Published: April 2004

Abstract
Highly ordered mesoporous Ni particles have been prepared by electroless deposition using lyotropic liquid crystals as templates. The bath conditions, in particular the kind of the reducing agents, greatly affected the degree of the ordering of mesostructures. By using well chosen appropriate reducing agents and combining those agents, we have succeeded in synthesizing Ni particles with highly ordered mesoporosity.
Preparation of Li1+y−xZnxMn2−yO4 Spinel as a Cathode Material for Li+-batteries
Authors: Noguchi Hideyuki , Nakamura Hiroyoshi , Yoshio Masaki , Wang Hongyu 
Published: April 2004

Abstract
Li1+y−xZnxMn2−yO4 spinel has been synthesized using LiOH, Zn(NO3)2, and MnO2. The substitution of Zn2+ for Li+ in the tetrahedral 8a site of spinel was successfully achieved by adjustment of the synthetic conditions, such as atmosphere and cooling rate. The performance of 8a site Zn-doped spinel was investigated in the terms of initial capacity and polarization.
Characterizing Clay Mineral Suspensions using Acoustic and Electroacoustic Spectroscopy — A Review
Authors: Guerin Marianne , Seaman John C. 
Published: April 2004

AbstractRecently, significant advances have been made in the theory and application of acoustic and electroacoustic spectroscopies for measuring the particle-size distribution (PSD) and zeta potential (ζ potential) of colloidal suspensions, respectively. These techniques extend or replace other techniques, such as light-scattering methods, particularly in concentrated suspensions. In this review, we summarize acoustic and electroacoustic theory and published results on clay mineral suspensions, detail theoretical constraints, and indicate potential applications for the study of environmentally significant clay mineral suspensions. Using commercially available instrumentation and suspension concentrations up to 45 vol.%, acoustic spectroscopy can characterize particle sizes from 10 nm to 10 µm, or greater. Electroacoustic spectroscopy can determine the ζ potential of a suspension with a precision and accuracy in the mV range. Despite the clear potential for their use in environmental settings, to date, acoustic methods have been used mainly on clay mineral colloids with industrial application, typically combined with similar measurements such as isoelectric point (IEP) determined from shear yield stress or ζ potential from electrophoretic mobility measurements. Potential applications in environmentally relevant suspension concentrations are significant, as PSD and ζ potential are important factors influencing the transport of mineral colloids and associated contaminants through porous media. Applications include determining the effects of suspension concentration, surfactants, electrolyte strength, pH and solution composition on soil clay properties and colloidal interactions, and determining changes in PSD, aggregation and ζ potential due to adsorption or variations in the clay mineralogy.
Evolutionärer Test des Zeitverhaltens von Realzeit-Systemen (Evolutionary Testing of the Temporal Behaviour of Real-Time Systems)
Authors: Wegener Joachim 
Published: April 2004

Zusammenfassung
Mit dem evolutionären Test steht eine neue Testmethode zur Verfügung, die eine systematische und weitgehend automatische Prüfung des Zeitverhaltens von Realzeit-Systemen ermöglicht. Der Test wird als ein Optimierungsproblem interpretiert, das mit evolutionären Algorithmen gelöst wird. Es werden die Eingabesituationen mit den längsten und kürzesten Ausführungszeiten gesucht, um dynamisch zu überprüfen, ob Verletzungen der für das zu testende Realzeit-System spezifizierten Zeitschranken auftreten.