bluuebunny/crossref_metadata_2025_split huggingface dataset:
A novel syndrome of variant leukocyte adhesion deficiency involving defects in adhesion mediated by β1 and β2 integrins
Authors: Harris Estelle S. , Shigeoka Ann O. , Li Wenhua , Adams Roberta H. , Prescott Stephen M. , McIntyre Thomas M. , Zimmerman Guy A. , Lorant Diane E. 
Published: July 2002

AbstractLeukocyte adhesion deficiency type I (LAD-1) is a disorder associated with severe and recurrent bacterial infections, impaired extravascular targeting and accumulation of myeloid leukocytes, altered wound healing, and significant morbidity that is caused by absent or greatly diminished surface expression of integrins of the β2 class. We report clinical features and analysis of functions of cells from a patient with a myelodysplastic syndrome and infectious complications similar to those in the severe form of LAD-1, but whose circulating neutrophils displayed normal levels of β2 integrins. Analysis of adhesion of these cells to immobilized ligands and to endothelial cells and assays of cell-cell aggregation and chemotaxis demonstrated a profound defect in adhesion mediated by β2 integrins indicative of a variant form of LAD-1. A novel cell line established from Epstein-Barr virus–transformed lymphoblasts from the subject demonstrated deficient β2 integrin–dependent adhesive function similar to that of the primary leukocytes. In addition, these cells had markedly impaired β1 integrin–dependent adhesion. Sequence analysis and electrophoretic mobility of β1 and β2 proteins from the cell line demonstrated that the defects were not a result of structural abnormalities in the integrin subunit chains themselves and suggest that the adhesive phenotype of these cells is due to one or more abnormalities of inside-out signaling mechanisms that regulate the activity of integrins of these classes. These features define a unique LAD-1 variant syndrome that may reveal important insights that are generally relevant to inside-out signaling of integrins, a molecular process that is as yet incompletely understood.
Inhibition of antigen-receptor signaling by Platelet Endothelial Cell Adhesion Molecule-1 (CD31) requires functional ITIMs, SHP-2, and p56lck
Authors: Newman Debra K. , Hamilton Christin , Newman Peter J. 
Published: July 2002

AbstractPlatelet Endothelial Cell Adhesion Molecule-1 (PECAM-1, CD31) is a 130-kd member of the immunoglobulin gene superfamily that is expressed on the surface of platelets, endothelial cells, myeloid cells, and certain lymphocyte subsets. PECAM-1 has recently been shown to contain functional immunoreceptor tyrosine-based inhibitory motifs (ITIMs) within its cytoplasmic domain, and co-ligation of PECAM-1 with the T-cell antigen receptor (TCR) results in tyrosine phosphorylation of PECAM-1, recruitment of Src homology 2 domain-containing protein tyrosine phosphatase-2 (SHP-2), and attenuation of TCR-mediated cellular signaling. To determine the molecular basis of PECAM-1 inhibitory signaling in lymphocytes, the study sought to (1) establish the importance of the PECAM-1 ITIMs for its inhibitory activity, (2) determine the relative importance of SHP-2 versus SHP-1 in mediating the inhibitory effect of PECAM-1, and (3) identify the protein tyrosine kinases required for PECAM-1 tyrosine phosphorylation in T cells. Co-ligation of wild-type PECAM-1 with the B-cell antigen receptor expressed on chicken DT40 B cells resulted in a marked reduction of calcium mobilization—similar to previous observations in T cells. In contrast, co-ligation of an ITIM-less form of PECAM-1 had no inhibitory effect. Furthermore, wild-type PECAM-1 was unable to attenuate calcium mobilization in SHP-2–deficient DT40 variants despite abundant levels of SHP-1 in these cells. Finally, PECAM-1 failed to become tyrosine phosphorylated in p56lck-deficient Jurkat T cells. Together, these data provide important insights into the molecular requirements for PECAM-1 regulation of antigen receptor signaling.
Neutrophil-specific granule deficiency: homozygous recessive inheritance of a frameshift mutation in the gene encoding transcription factor CCAAT/enhancer binding protein–ε
Authors: Gombart Adrian F. , Shiohara Masaaki , Kwok Scott H. , Agematsu Kazunaga , Komiyama Atsushi , Koeffler H. Phillip 
Published: July 2002

Abstract
Neutrophil-specific granule deficiency (SGD) is a rare congenital disorder. The neutrophils of individuals with SGD display atypical bi-lobed nuclei, lack expression of all secondary and tertiary granule proteins, and possess defects in chemotaxis, disaggregation, receptor up-regulation, and bactericidal activity, resulting in frequent and severe bacterial infections. Previously, a homozygous mutation in theCCAAT/enhancer binding protein–ε (C/EBPε) gene was reported for one case of SGD. To substantiate the role of C/EBPε in the development of SGD and elucidate its mechanism of inheritance, the mutational status of the gene was determined in a second individual. An A-nucleotide insertion in the coding region of the C/EBPε gene was detected. This mutation completely abolished the predicted translation of all C/EBPε isoforms. Microsatellite and nucleotide sequence analyses of the C/EBPε locus in the parents of the proband indicated that the disorder may have resulted from homozygous recessive inheritance of the mutant allele from an ancestor shared by both parents. The mutant C/EBPε32 protein localized in the cytoplasm rather than the nucleus and was unable to activate transcription. Consistent with this, a significant decrease in the levels of the messenger RNAs (mRNAs) encoding the secondary granule protein human 18-kd cationic antimicrobial protein (hCAP-18)/LL-37 and the primary granule protein bactericidal/permeability-increasing protein were observed in the patient. The hCAP-18 mRNA was induced by overexpression of C/EBPε32 in the human myeloid leukemia cell line, U937, supporting the hypothesis that C/EBPε is a key regulator of granule gene synthesis. This study strongly implicates mutation of theC/EBPε gene as the primary genetic defect involved in the development of neutrophil SGD and defines its mechanism of inheritance.
Preferential and persistent depletion of CCR5+T-helper lymphocytes with nonlymphoid homing potential despite early treatment of primary HIV infection
Authors: Krzysiek Roman , Rudent Annick , Bouchet-Delbos Laurence , Foussat Arnaud , Boutillon Claudie , Portier Alain , Ingrand Didier , Sereni Daniel , Galanaud Pierre , Grangeot-Keros Liliane , Emilie Dominique ,   the ANRS O86 PRIMOFERON A Study Group
Published: July 2002

AbstractStrains of human immunodeficiency virus (HIV) transmitted between individuals use the CCR5 coreceptor, but no preferential depletion of particular Th-lymphocyte subpopulations has been reported during primary HIV infection (PHI). In contrast, gut-associated Th lymphocytes are preferentially depleted in macaques recently infected by simian immunodeficiency virus. The expression of CCR5 and the intestinal homing receptor integrin α4β7 on subpopulations of Th lymphocytes was studied in 12 patients with PHI. There was a profound decrease of circulating α4β7+ Th lymphocytes and CCR5+ memory Th lymphocytes with nonlymphoid homing potential (CD62L−CD45RO+). Unlike other Th lymphocytes, this cell population remained depleted despite early control of viral replication under antiretroviral treatment. Therefore, HIV preferentially targets a specific CCR5+ subpopulation of Th lymphocytes early during infection, inducing its persistent depletion despite treatment. Protective immunity in vivo depends on Th lymphocytes carrying homing capacity to nonlymphoid tissue, and therefore these data may explain the persistent abnormalities of immune functions in patients infected with HIV.
Interaction of calmodulin with the cytoplasmic domain of the platelet membrane glycoprotein Ib-IX-V complex
Authors: Andrews Robert K. , Munday Adam D. , Mitchell Christina A. , Berndt Michael C. 
Published: July 2002

AbstractEngagement of platelet membrane glycoprotein (GP) Ib-IX-V by von Willebrand factor triggers Ca++-dependent activation of αIIbβ3, resulting in (patho)physiological thrombus formation. It is demonstrated here that the cytoplasmic domain of GPIb-IX-V associates with cytosolic calmodulin. First, an anti-GPIbα antibody coimmunoprecipitated GPIb-IX and calmodulin from platelet lysates. Following platelet stimulation, calmodulin dissociated from GPIb-IX and, like the GPIb-IX–associated proteins 14-3-3ζ and p85, redistributed to the activated cytoskeleton. Second, a synthetic peptide based on the cytoplasmic sequence of GPIbβ, R149–L167 (single-letter amino acid codes), affinity-isolated calmodulin from platelet cytosol in the presence of Ca++ as confirmed by comigration with bovine calmodulin on sodium dodecyl sulfate–polyacrylamide gels, by sequence analysis, and by immunoreactivity with the use of an anticalmodulin antibody. The membrane-proximal GPIbβ sequence was analogous to a previously reported calmodulin-binding sequence in the leukocyte adhesion receptor, L-selectin. In addition, the cytoplasmic sequence of GPV, K529–G544, was analogous to a calmodulin-binding IQ motif within the α1c subunit of L-type Ca++ channels. Calmodulin coimmunoprecipitated with GPV from resting platelet lysates, but was dissociated in stimulated platelets. A GPV-related synthetic peptide also bound calmodulin and induced a Ca++-dependent shift on nondenaturing gels. Together, these results suggest separate regions of GPIb-IX-V can directly bind calmodulin, and this novel interaction potentially regulates aspects of GPIb-IX-V–dependent platelet activation.
Lentiviral gene transfer into peripheral blood–derived CD34+ NOD/SCID-repopulating cells
Authors: Scherr Michaela , Battmer Karin , Blömer Ulrike , Schiedlmeier Bernd , Ganser Arnold , Grez Manuel , Eder Matthias 
Published: July 2002

Abstract
This study reports a lentiviral gene transfer protocol for efficient transduction of adult human peripheral blood (PB)–derived CD34+ NOD/SCID-repopulating cells (SRCs) using vesicular stomatitis virus–G protein (VSV-G)–pseudotyped lentiviruses encoding for enhanced green fluorescence protein (eGFP). Lentiviral stocks were concentrated by anion exchange chromatography, and transduction was performed under serum-free conditions at a multiplicity of infection (MOI) between 3 and 50. Similar transduction efficiencies were achieved in the presence and absence of cytokines. Transduction of PB-derived CD34+cells at a MOI of 3 resulted in gene transfer efficiencies into SRCs of 9.2% and 12.0% in the absence and presence of cytokines, respectively. Using improved lentiviral vectors, transduction frequency varied between 42.0% (MOI 10) and 36.0% (MOI 50) with multilineage transgene expression within SRC-derived myeloid and lymphoid cells. The protocol described can be adapted for clinical application of lentiviral gene transfer into PB-derived CD34+ cells from adult patients.
Long-Term Medical Outcomes and Quality-of-Life Assessment of Patients With Chronic Myeloid Leukemia Followed at Least 10 Years After Allogeneic Bone Marrow Transplantation
Authors: Kiss T.L. , Abdolell M. , Jamal N. , Minden M.D. , Lipton J.H. , Messner H.A. 
Published: July 2002

PURPOSE: Benchmark analysis of patients with chronic myeloid leukemia (CML) alive for more than 10 years after allogeneic bone marrow transplantation (BMT) including data on disease status, bone marrow reserve, long-term complications, and quality of life (QOL). PATIENTS AND METHODS: Eighty-nine patients (46 in first chronic phase, 43 in advanced phase) received an allogeneic BMT for CML during the study period. Medical outcomes and QOL of patients were analyzed retrospectively. RESULTS: Twenty-eight (31.5%) of 89 patients were alive at 10 years and included in this analysis. Thirteen (46.4%) of 28 long-term survivors never relapsed. Fifteen patients relapsed between 0.5 and 16 years after transplantation. Ten patients showed a hematologic relapse and received salvage treatment. Five patients showed transient low levels of BCR-ABL–positive cells by Southern blot with no subsequent hematologic relapse. One of the 28 patients died in blast crisis at 12 years. The most frequent long-term complications were chronic graft-versus-host disease, osteoporosis, and cataracts. Frequency of clonogenic progenitors remained persistently decreased. QOL assessment yielded lower scores in physical performance as compared with an age-matched normative population, whereas social functioning was equivalent. A high degree of satisfaction was noted with interpersonal relationships. CONCLUSION: Patients with CML surviving their BMT long term do well in terms of medical outcomes. A constant rate of relapse was noted, with a high salvage rate of affected patients, suggesting the need for lifelong monitoring. QOL is perceived as good, particularly as related to social functioning; however, it is inferior to a normative population with regard to physical performance.
Racial Disparities and Achievement of the Low Lupus Disease Activity State: A CARRA Registry Study
Authors: Soulsby William Daniel , Olveda Rebecca , He Jie , Berbert Laura , Weller Edie , Barbour Kamil E. , Greenlund Kurt J. , Schanberg Laura E. , von Scheven Emily , Hersh Aimee , Son Mary Beth F. , Chang Joyce , Knight Andrea ,   the CARRA Registry Investigators
Published: September 2024

ObjectiveDifferential disease control may contribute to racial disparities in outcomes of childhood‐onset systemic lupus erythematosus (cSLE). We evaluated associations of race and individual‐ or neighborhood‐level social determinants of health (SDoH) with achievement of low lupus disease activity state (LLDAS), a clinically relevant treatment target.MethodsIn this cSLE cohort study using the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry, the primary exposure was self‐reported race and ethnicity, and collected SDoH included insurance status and area deprivation index (ADI). Outcomes included LLDAS, disease activity, and time‐averaged prednisone exposure. Associations among race and ethnicity, SDoH, and disease activity were estimated with multivariable regression models, adjusting for disease‐related and demographic factors.ResultsAmong 540 children with cSLE, 27% identified as Black, 25% identified as White, 23% identified as Latino/a, 11% identified as Asian, 9% identified as more than one race, and 5% identified as other. More Black children (41%) lived in neighborhoods of highest ADI compared to White children (16%). Black race was associated with lower LLDAS achievement (adjusted odds ratio 0.56, 95% confidence interval [CI] 0.38–0.82) and higher disease activity (adjusted β 0.94, 95% CI 0.11–1.78). The highest ADI was not associated with lower LLDAS achievement on adjustment for renal disease and insurance. However, renal disease was found to be a significant mediator (P = 0.04) of the association between ADI and prednisone exposure.ConclusionsChildren with cSLE who identified as Black are less likely to achieve LLDAS and have a higher disease activity. Living in areas of higher ADI may relate to renal disease and subsequent prednisone exposure. Strategies to address root causes will be important to design interventions mitigating cSLE racial disparities.
Multifunctional Ammonium Sulfide Enables Highest Efficiency Lead–Tin Perovskite Solar Cells
Authors: Song Jing , Kong Tengfei , Zhao Zihan , Zhang Yang , Liu Yinjiang , Bi Dongqin 
Published: September 2024

AbstractThe commonly used hole transport layer (HTL) Poly(3,4‐ethylenedioxythiophene):poly(styrene sulfonate) (PEDOT:PSS) still has some shortcomings that will limit the development of Pb–Sn perovskite solar cells (PSCs). In this work, multifunctional ammonium sulfide (AS) is attempted to modify PEDOT:PSS/perovskite interface. AS has multiple effects on different functional layers and the device as a whole: a) optimize the perovskite crystallization by the formation of PbS nucleation sites; b) neutralize the acidity of PEDOT:PSS by reaction with PSS; c) promote carrier transport at HTL/perovskite interface by tuning the energy level of PEDOT:PSS. As a result, a power conversion efficiency of 24.23% is obtained in the reverse scanning direction and 23.84% in the steady‐state output power test for the single‐junction Pb–Sn PSCs, and 27.48% for all‐perovskite tandem solar cells. These results show that AS modification can be an effective way to boost the development of Pb–Sn PSCs.
Advanced Dual‐Band Smart Windows: Inorganic All‐Solid‐State Electrochromic Devices for Selective Visible and Near‐Infrared Modulation
Authors: Chen Mingjun , Deng Jianbo , Zhang Hulin , Zhang Xiang , Yan Dukang , Yao Gengxin , Hu Liping , Sun Shuokun , Zhao Jiupeng , Li Yao 
Published: September 2024

AbstractDual‐band electrochromic smart windows (DESWs), capable of actively and selectively modulate visible (VIS) light and near‐infrared (NIR) heat, have emerged as a practical technology for enhancing user comfort and reducing building energy consumption. However, the design and development of DESWs remain a significant challenge due to the difficulty in obtaining suitable materials and high‐durability electrolytes. Here, the first all‐solid‐state DESW based on an orthorhombic oxygen‐deficient tungsten oxide (o‐WO3‐x) film is presented. Benefiting from the synergistic effects of the efficient oxygen‐vacancy‐enhanced charge transfer process and the secure transfer pathway enabled by the orthorhombic crystal structure, the o‐WO3‐x film showcases remarkable dual‐band electrochromic properties, including selective modulation of VIS light and NIR heat, large optical modulation (89.1%), rapid response time (tb/tc = 6.8/17.9 s), high coloration efficiency (155.92 cm2 C−1), and ultrastable cyclic performance (8000 cycles) even in acidic aqueous electrolyte. Furthermore, the all‐solid‐state DESWs incorporating o‐WO3‐x deliver a significant and stable dual‐band electrochromic response with excellent thermal regulation and energy‐saving capabilities. These findings underscore the considerable potential of o‐WO3‐x films and their all‐solid‐state smart windows in decreasing building energy consumption.
Crystalline Orientation Control Enhanced Adhesion of Perovskite to Large‐Area Readout Board for High‐Performance X‐Ray Imaging
Authors: Qian Wei , Huang Duan , Tang Yupu , Yang Wanshun , Li Han , Lei Renbo , Men Haoyu , Wang Xinwei , Yang Shihe 
Published: September 2024

AbstractHalide perovskites are reputed as highly promising photoelectronic materials for direct X‐ray detectors, but realizing large‐area flat‐panel imaging requires to address the compatibility issue of the electronic, surficial, and mechanical properties between the perovskite and the readout circuit board. Here, a low‐dimensional MA3Bi2I9 perovskite is chosen to achieve a good match in a balancing act between the two by exploiting an orientation control strategy for perovskite film growth. The most striking consequence of the orientation controlled growth is the excellent adhesion of the thick perovskite film to the electronic board in large area and effectively addresses the charge sharing effect, which has been notoriously difficult to achieve. The resulting detector, exhibits an X‐ray imaging area of 2.8 × 3.2 cm, with a spatial resolution of 4.0 lp mm⁻¹, the highest yet achieved for polycrystalline perovskite detectors based on TFT backplanes, and a sensitivity of 588 µCGyair−1 cm−2 while maintaining a dark current below 10 nA cm⁻2, this is also the highest value recorded to date for polycrystalline zero‐dimensional perovskite detectors. This device clearly revealing the intricate internal structures of both biological specimens and industrial products. This outcome demonstrates the potential of zero‐dimensional perovskites in X‐ray planar imaging and highlights the critical role of orientation control strategies.
Constructing LiF‐Enriched Solid Electrolyte Interface on Graphene Arrays with Abundant Edges on Microscale Si‐C Anodes Toward High‐Energy Lithium‐Ion Batteries
Authors: Ge Ke , Wang Zhenhong , Liu Jie , Mu Yongbiao , Wang Rui , Xu Xiaoqian , Wang Yichun , Zou Zhiyu , Zhang Qing , Han Meisheng , Zeng Lin 
Published: September 2024

AbstractSilicon (Si) anodes offer excellent lithium storage capacity for lithium‐ion batteries but face practical limitations due to significant volume expansion and low intrinsic electrical conductivity. These issues lead to side reactions that consume the electrolyte and impede ion‐electron transport, resulting in low areal loading (<2 mg cm⁻²) and restricted energy density. To address this, a scalable method is developed using spray drying of commercial graphite flakes (s‐Gr) and nanosilicon particles (n‐Si), followed by chemical vapor deposition to create microscale Si/C anodes (s‐Gr/n‐Si/VGs). Thin vertical graphene nanosheets (VGs) are grown on the surfaces and within the internal pores, forming a robust, micron‐sized Si/C spherical composite material. The VGs construct the conductive network, allowing the electrodes to operate at high areal loadings without pulverization and promoting LiF‐enriched solid electrolyte interphase for improved cycling stability. The s‐Gr/n‐Si/VGs maintain a capacity of 641.9 mAh g⁻¹ after 1000 cycles at 11.0 mg cm⁻², retaining 95.9% capacity. In pouch cells with NCM811 cathodes, the 5.0 Ah‐level cells achieved 80.0% capacity retention after 510 cycles at 1.0 C. This research provides a feasible pathway for manufacturing high‐performance, low‐cost, and scalable Si/C anodes suitable for high‐energy‐density lithium‐ion batteries.
Lignin‐Based Macromolecular Photoinitiator for Non‐Migration, Self‐Adhesive, and Water‐Resistant Eutectogels toward Underwater Reliable Communication
Authors: Luo Tong , Qi Ji , Yu Juan , Wang Chunpeng , Chu Fuxiang , Wang Jifu 
Published: September 2024

AbstractHydrophobic conductive gels have attracted much attention in underwater sensing as the soft robots and wearable sensors. Among the various methods for preparing conductive gels, UV‐initiated polymerization shows advantages of rapid curing and significant energy savings. However, traditional commonly used photoinitiators in UV‐initiated polymerization often migrate or diffuse within conductive gels, especially with prolonged use or underwater, posing potential environmental and health risks. Herein, a novel lignin‐based macromolecular photoinitiator (L‐BF) is first designed by incorporating benzoylformate into the lignin backbone, demonstrating excellent UV absorption, high radical initiation efficiency, and hydrophobicity. Subsequently, hydrophobic eutectogels (HEG) are prepared via UV‐initiated polymerization using L‐BF as photoinitiator. The HEG shows excellent mechanical properties (toughness of 5.67 MJ m−3 and strain of 941%) and scarcely L‐BF migration underwater. Moreover, the hydrophobic network of L‐BF endowed HEG with hydrophobicity (water contact angle exceeds 110°; swelling ratio is <5.8%), superior underwater adhesion (255 kPa), even maintained excellent sensing performance (gauge factor of 4.69), and long‐term reproducibility (20 000 cycles) in air and underwater. Validation confirms the potential of HEG as an effective wearable sensor for underwater posture monitoring and communication, advancing the use of lignin and low‐migration conductive gels in marine exploration.