bluuebunny/crossref_metadata_2025_split huggingface dataset:
Two siblings with homocystinuria presenting with dystonia and parkinsonism
Authors: Ekinci Burçak , Apaydin Hulya , Vural Melih , Özekmekçi Sibel 
Published: April 2004

AbstractMovement disorders such as dystonia, chorea or tremor are rarely encountered in patients with homocystinuria. We present 2 siblings with laboratory‐confirmed homocystinuria, one with severe generalized dystonia and the other with mild parkinsonism. The movement disorders in our patients appeared in the second and first decades, respectively. © 2004 Movement Disorder Society
Parkinson's disease‐like presentation of multiple system atrophy with poor response to STN stimulation: A clinicopathological case report
Authors: Lezcano Elena , Gómez‐Esteban Juan C. , Zarranz Juan J. , Alcaraz Rafael , Atarés Begoña , Bilbao Gaizka , Garibi Jesús , Lambarri Imanol 
Published: April 2004

AbstractWe report on a clinicopathological case of multiple system atrophy with good response to levodopa and subsequent development of motor complications. Because the subject complied with all the inclusion criteria (Core Assessment Program for Surgical Interventional Therapies in Parkinson's Disease), bilateral subthalamic nucleus deep brain stimulation electrodes were implanted. © 2004 Movement Disorder Society
Fracture of the odontoid process complicating tardive dystonia
Authors: Konrad Carsten , Vollmer‐Haase Juliane , Gaubitz Markus , Nabavi Darius G. , Reilmann Ralf , Knecht Stefan 
Published: April 2004

AbstractWe report on a 57‐year‐old woman with frequent and powerful retrocollis. The involuntary movements combined with rheumatoid arthritis and osteoporosis led to a fracture of the axial odontoid process. Neurologists with a focus on movement disorders should screen for diseases putting the patient at risk for spinal lesions and consider early treatment. © 2004 Movement Disorder Society
Adrafinil‐induced orofacial dyskinesia
Authors: Thobois Stéphane , Xie Jing , Mollion Helena , Benatru Isabelle , Broussolle Emmanuel 
Published: April 2004

AbstractWe describe the first case of orofacial abnormal movements induced by adrafinil, a vigilance promoting agent of the same pharmacological class as modafinil. The dyskinesias did not spontaneously recover despite adrafinil withdrawal for a 4‐month period. They were secondly dramatically improved by tetrabenazine, a presynaptic dopaminergic depleting drug which was introduced after the 4‐month adrafinil‐free period. © 2004 Movement Disorder Society
Cyclooxygenase and 5‐lipoxygenase pathways in the vessel wall: Role in atherosclerosis
Authors: Vila Luís 
Published: April 2004

AbstractProstaglandins (PGs), thromboxanes (Txs), and leukotrienes (LTs) play a relevant role in cardiovascular physiology and pathophysiology. Recent reports concerning cardiovascular risk associated with cyclooxygenase‐2 selective inhibitors have prompted questions about the “protective” or “deleterious” role of each COX isoform in cardiovascular disease, and the cloning and expression of inducible PGE‐synthase (PGES) open the possibility that PGES could be a new therapeutical target in this context. Predominance of constricting or relaxing prostanoids depends not only on COX activity but also to downstream enzymes such as PGI‐synthase (PGIS) and PGES. In the vessel wall, PGIS and PGES seem to be major downstream enzymes in the endothelium and smooth muscle, respectively. Like COX, activity of these enzymes can also be regulated by several factors, which include nitrogen oxides, cytokines, and lipid peroxides. LTs are important inflammatory mediators also involved in the pathophysiology of cardiovascular disease, which are targets for pharmacological intervention. Unlike COX pathway, the biosynthesis of chemotactic and vaso‐constrictor LTs in the vasculature strongly depends on leukocyte recruitment and activation, and on cell‐cell interaction between leukocytes and vascular cells in the inflamed areas. The present review emphasizes the role of vascular‐derived prostanoids and LTs on atherosclerosis. © 2004 Wiley Periodicals, Inc. Med Res Rev, 24, No. 4, 399–324, 2004
Governance for sustainable poverty reduction: the social fund in Nicaragua
Authors: Dijkstra A. Geske 
Published: April 2004

AbstractLike other social funds, the Nicaraguan Emergency Social Investment Fund (FISE) has been created on the initiative of the donors to alleviate the social consequences of adjustment policies. It finances small infrastructural projects in education, health, water and other sectors and focuses on poor communities. FISE accounts for approximately 11% of public investment and 48% of public investment in the social sectors. The size of FISE and its apparent permanent character raise questions of its contribution to sustainable poverty reduction and on institutional development in the country. The article concludes that there are serious doubts on whether maintaining a social fund as a separate agency enhances effective governance for sustainable poverty reduction. Copyright © 2004 John Wiley & Sons, Ltd.
Scaling‐up participation at USAID
Authors: Corneille Faith , Shiffman Jeremy 
Published: April 2004

AbstractSince the 1970s many non‐governmental development organisations have moved away from top‐down modes of operation toward participatory practices that hand over decision‐making power to the poor. The bilateral development agencies of the industrialised states have been slower to follow suit, but in the 1980s and 1990s a number of such agencies also initiated participatory‐oriented reforms. Development scholars have paid little attention to reforms of the larger organisations. In this article, we analyse the efforts of the United States Agency for International Development to embrace participation in the 1990s. Internal reformers managed to change some agency procedures but had only limited success in institutionalising participatory practices. Forces internal to the agency, including rigid rules and employee incentive structures, hampered reform efforts. Also, while some developments external to the agency facilitated participation, many others, including legislative interference and shifting national policy priorities, hindered change toward participation. The case reveals how complex webs of accountability relationships make participatory‐oriented transformation a cumbersome process in large development organisations. Change towards participation can succeed, but only if reformers acknowledge and consider existing institutional constraints. Copyright © 2004 John Wiley & Sons, Ltd.
Allelic length of a CA dinucleotide repeat in the egfr gene correlates with the frequency of amplifications of this sequence—first results of an inter‐ethnic breast cancer study
Authors: Buerger Horst , Packeisen Jens , Boecker Almuth , Tidow Nicola , Kersting Christian , Bielawski Krzysztof , Isola Jorma , Yatabe Yasushi , Nakachi Kei , Boecker Werner , Brandt Burkhard 
Published: April 2004

AbstractOverexpression of the epidermal growth factor receptor (EGFR) is a common finding in invasive breast cancer and represents a potential target for new treatment options. However, little is known about the parameters that might indicate a potential clinical response for these anti‐EGFR‐based therapies. In order to gain further insights into the interplay between the length of a CA‐SSR I repeat in intron 1 of egfr, copy numbers of this untranslated regulatory sequence, and protein expression, the present study investigated breast cancers from Germans and Japanese patients by microsatellite analysis, quantitative 5′ nuclease assay by egfr enzyme‐linked immunosorbent assay (ELISA), and comparative genomic hybridization (CGH). Japanese breast cancer patients displayed significantly longer alleles for the CA‐SSR I repeat (p < 0.001), associated with significantly lower EGFR expression (mean 65 versus 36 fmol/mg membrane protein). Allelic imbalance (restricted to CA‐SSR I) was observed in 55% of the informative Japanese breast cancers compared with only 34% of the German breast cancer reference group. Using a quantitative 5′ nuclease assay for egfr, a significantly higher percentage of Japanese breast cancer patients revealed amplifications of the CA‐SSR I repeat (p < 0.01). Japanese patients with these amplifications were characterized by a significantly higher EGFR content compared with the German breast cancer patients (p < 0.05). These data show, on the one hand, that the correlation of EGFR overexpression and an inherited CA repeat polymorphism within intron 1 of egfr is a general finding in breast cancer, as has been shown previously. On the other hand, the data demonstrate clearly for the first time an interaction between the length of a polymorphism in intron 1 of egfr as an inherited genetic factor and the frequency of egfr amplification, as an acquired genetic factor, both factors contributing to EGFR overexpression in breast cancer. This new knowledge about mechanisms of regulation of EGFR expression might serve as an additional basis for evaluating anti‐EGFR‐based therapies. Copyright © 2004 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Expression of regulator of G protein signalling protein 5 (RGS5) in the tumour vasculature of human renal cell carcinoma
Authors: Furuya Mitsuko , Nishiyama Mariko , Kimura Sadao , Suyama Takahito , Naya Yukio , Ito Haruo , Nikaido Takashi , Ishikura Hiroshi 
Published: April 2004

AbstractThe gene expression profiles of tumour and normal vasculature are distinctively different. The altered expression of various angiogenesis‐related genes in tumour‐derived endothelial cells has been investigated intensively, but there may be as yet unidentified molecules that regulate tumour angiogenesis. In the present study, the distinctive expression of regulator of G protein signalling protein 5 (RGS5) in tumour vessels in human renal cell carcinoma (RCC) has been clarified. RGS5 is a member of the RGS superfamily and acts as a negative regulator of heterotrimeric G protein‐mediated signalling through G protein‐coupled receptors (GPCRs). RT‐PCR showed strong expression of RGS5 in all RCCs examined, but expression was very weak or undetectable in normal kidneys. By real‐time RT‐PCR, the ratio of RGS5 mRNA in RCC to that in normal kidney was 6.6 : 1 (p = 0.0012). In situ hybridization showed strong expression of RGS5 in vessels within tumour cell nests. It was expressed neither in tumour cells nor in normal renal capillaries. Immunohistochemical staining using serial sections for endothelial cell markers (CD31 and CD34) and smooth muscle cell markers (α‐SMA and desmin), as well as fluorescence double staining, strongly suggested that tumour endothelial cells were the main location of RGS5 in RCC. These findings suggest that RGS5 may be involved in G protein‐mediated signalling in tumour vessels in human RCC. Copyright © 2004 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Type I and III collagen protein precursors and mRNA in the developing human lung
Authors: Kaarteenaho‐Wiik Riitta , Pääkkö Paavo , Herva Riitta , Risteli Juha , Soini Ylermi 
Published: April 2004

AbstractExtracellular matrix proteins have a prominent role in both ontogenesis and fibrogenesis in the human lung. The aim of this study was to analyse the expression of newly formed precursor proteins and mRNA of collagen types I and III in developing human lung tissues from 12 to 40 weeks of gestation, and also in neonatal disorders such as respiratory distress syndrome (RDS) and bronchopulmonary dysplasia (BPD). Lung tissues were obtained at autopsy from 60 non‐malformed cases. All tissues were analysed by immunohistochemistry and 24 were also investigated by mRNA in situ hybridization. The precursor proteins and mRNA of both collagens were expressed in abundance in pulmonary arteries and veins during all developmental periods. In RDS and BPD, precursor proteins and mRNAs of both collagen types were increased within alveolar walls. The cells in these locations showed α‐smooth muscle actin, vimentin, and variable desmin immunoreactivity. Collagen I and III precursor proteins and mRNA were also observed in pleura, bronchi, bronchioles, and around chondrocytes during all developmental periods and in diseased lung. In conclusion, collagens I and III were expressed in a similar way in and around various cell types in the developing lung and their expression was increased within alveolar walls in RDS and BPD. Myofibroblast‐type cells appeared to produce mRNA for both types of collagen in alveoli. Copyright © 2004 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Remodelling of the pulmonary arteries during recovery from pulmonary hypertension induced by neonatal hypoxia
Authors: Hall Susan M , Hislop Alison A , Wu Zhongshi , Haworth Sheila G 
Published: April 2004

AbstractLittle is understood of the mechanisms involved in reducing pulmonary arterial wall thickness on recovery from pulmonary hypertension and the present study sought to clarify the events that occur. Piglets were exposed to hypobaric hypoxia for 3 days, either from birth or from 3 days of age, and others were exposed for 11 days starting at 3 days. All recovered in room air for up to 6 days. Using light and electron microscopy, the pulmonary artery wall thickness, the relative contribution of smooth muscle and matrix, smooth muscle cell replication, and apoptosis were assessed after hypoxic exposure and during recovery from hypoxic exposure. In elastic arteries, after 6 days' recovery in room air, a reduction in wall thickness to normal was associated with a similar reduction in proportional area of smooth muscle cells and matrix (p < 0.05), increased apoptosis (p < 0.05), and an abnormally low replication rate (p < 0.05). In peripheral muscular arteries, an increase in external diameter, and wall thinning on recovery, was achieved by smooth muscle cell remodelling and a reduction in cell replication (p < 0.05). Apoptosis did not contribute. Thus, different mechanisms are involved in recovery from hypoxia‐induced pulmonary hypertension in elastic and muscular pulmonary arteries. Recovery is slower in animals exposed from birth rather than from 3 days of age. Copyright © 2004 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Properties of SEPT9 isoforms and the requirement for GTP binding
Authors: Robertson Claire , Church Stewart W , Nagar Hans A , Price John , Hall Peter A , Russell SE Hilary 
Published: April 2004

AbstractMembers of the evolutionarily conserved septin family of genes are emerging as key components of several cellular processes including membrane trafficking, cytokinesis, and cell‐cycle control events. SEPT9 has been shown to have a complex genomic architecture, such that up to 15 different isoforms are possible by the shuffling of five alternate amino termini and three alternate carboxy termini. Genomic and transcriptional alterations of SEPT9 have been associated with neoplasia. The present study has used a Sept9‐specific antibody to determine the pattern of isoform expression in a range of tumour cell lines. Western blot analysis indicated considerable variation in the relative amounts and isoform content of Sept9. Immunofluorescence studies showed a range of patterns of cytoplasmic localization ranging from mainly particulate to mainly filamentous. Expression constructs were also generated for each amino terminal isoform to investigate the patterns of localization of individual isoforms and the effects on cells of ectopic expression. The present study shows that the epsilon isoform appears filamentous in this overexpression system while the remaining isoforms are particulate and cytoplasmic. Transient transfection of individual constructs into tumour cell lines results in cell‐cycle perturbation with a G2/M arrest and dramatic growth suppression, which was greatest in cell lines with the lowest amounts of endogenous Sept9. Similar phenotypic observations were made with GTP‐binding mutants of all five N‐terminal variants of Sept9. However, dramatic differences were observed in the kinetics of accumulation of wild‐type versus mutant septin protein in transfected cells. In conclusion, the present study shows that the expression patterns of Sept9 protein are very varied in a panel of tumour cell lines and the functional studies are consistent with a model of septin function as a component of a molecular scaffold that contributes to diverse cellular functions. Alterations in the levels of Sept9 protein by overexpression of individual isoforms can clearly perturb cellular behaviour and may thus provide a mechanistic explanation for observations of deranged septin expression in neoplasia. Copyright © 2004 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Caveolins as tumour markers in lung cancer detected by combined use of cDNA and tissue microarrays
Authors: Wikman Harriet , Seppänen Jouni K , Sarhadi Virinder K , Kettunen Eeva , Salmenkivi Kaisa , Kuosma Eeva , Vainio‐Siukola Katri , Nagy Balint , Karjalainen Antti , Sioris Thanos , Salo Jarmo , Hollmén Jaakko , Knuutila Sakari , Anttila Sisko 
Published: April 2004

AbstractTo identify new potential diagnostic markers for lung cancer, the expression profiles of 37 lung tumours were analysed using cDNA arrays. Seven samples were from small‐cell lung cancer (SCLC), two from large‐cell neuroendocrine tumours (LCNEC), and 28 from other non‐small‐cell lung cancers (mainly squamous cell cancer and adenocarcinoma). Principal component analysis and the permutation test were used to detect differences in the gene expression profiles and a set of genes was found that distinguished high‐grade neuroendocrine carcinomas (SCLC and LCNEC) from other lung cancers. In addition, several genes, such as caveolin‐1 (CAV1) and caveolin‐2 (CAV2), were constantly deregulated in all types of tumour sample, compared with normal tissue. The expression of these two genes was investigated further at the protein level on a tissue microarray containing tumours from 161 patients and normal tissues. Immunostaining for CAV1 was negative in 48% of tumours, whereas 28% of the tumours did not express CAV2. Lack of CAV1 protein expression was not caused by methylation or mutation. In stage I adenocarcinomas, CAV2 protein expression correlated with shorter survival. In conclusion, the present study was able to identify genes that have not previously been implicated in lung cancer by the combined use of two different array techniques. Some of these genes may provide novel diagnostic markers for lung cancer. Copyright © 2004 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Prion protein is ubiquitinated after developing protease resistance in the brains of scrapie‐infected mice
Authors: Kang Shin‐Chung , Brown David R , Whiteman Matthew , Li Ruliang , Pan Tao , Perry George , Wisniewski Thomas , Sy Man‐Sun , Wong Boon‐Seng 
Published: April 2004

AbstractAlthough the key event in the pathology of prion diseases is thought to be the conversion of cellular prion protein (PrPC) to the protease‐resistant scrapie species termed PrPSc, the factors that contribute to neurodegeneration in scrapie‐infected animals are poorly understood. One probable determinant could be when the accumulation of PrPSc in infected brain overwhelms the ubiquitin–proteasome system and triggers the degenerative cascade. In the present study, it was found that in mouse brains infected with the ME7 scrapie strain, the level of ubiquitin protein conjugates increased significantly at ∼144 days post‐infection (pi) when clinical signs first become apparent. This elevation correlated with the detection of protease‐resistant PrPSc and a decline in two endopeptidase activities associated with proteasome function. However, ubiquitination of PrP was only detected at the terminal stage, 3 weeks after the development of clinical symptoms (∼165 days pi). These results suggest that ubiquitination of PrP is a late event phenomenon and this conjugation occurs after the formation of protease‐resistant PrPSc. Whether this post‐translational modification and the impairment of proteasome function are pivotal events in the pathogenesis of prion diseases remains to be determined. Copyright © 2004 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Attenuating PP/PET bicomponent melt blown microfibers
Authors: Zhao Rongguo , Wadsworth Larry C. 
Published: April 2004

AbstractThis research investigated the attenuation of polypropylene (PP)/poly(ethylene terephthalate) (PET) bicomponent (bico) filaments during the melt blowing (MB) process. It was found that both mono‐ and bi‐component filaments attenuated from several hundred micrometers to a few micrometers in the first 5 centimeters from the die. However, fiber diameter distributions were found to be broad in these regions. The filaments were attenuated much slower but exhibited narrower diameter distributions as they moved further from the die. The diameters of bico MB filaments were between those of 100% PP and 100% PET filaments. The PET component in a bico filament controls the final fiber diameter. During melt blowing, filaments were aligned orderly with the airflow direction in a short distance near the die. Filament entanglements started at about 2.5 cm from the die and became more and more randomly oriented as the distance‐from‐the‐die (DFD) increased. The fiber diameter distribution of bico filaments was broader than that of 100% PP filaments. A higher airflow rate led to a narrower fiber size distribution for bico filaments.
Mechanical behavior of a semicrystalline polymer before necking. Part II: Modeling of uniaxial behavior
Authors: Brusselle‐Dupend N. , Lai D. , Feaugas X. , Guigon M. , Clavel M. 
Published: April 2004

AbstractBased on the experimental mechanical characterization presented in Part I of this paper, a model allowing the prediction of the behavior of the studied semicrystalline polymer in the pre‐necking domain was constructed. The modeling of the viscoelastoplastic behavior of polypropylene (PP) was described by partitioning the total strain into a viscoelastic strain and a viscoplastic strain. After being improved with experimental observations, the rheological model of Zener was used to model the viscoelastic behavior of PP. As for viscoplastic behavior modeling, it was based on the characterization of the mechanical behavior performed in the first part of the study. Attention was focused on the strain reversal in order to predict the unloading path. Experimental data from the performed mechanical tests were employed to determine the parameters of the viscoelastoplastic model.
Microcellular model evaluation for the deformation of dynamically vulcanized EPDM/iPP blends
Authors: Wright Kathryn J. , Indukuri Kishore , Lesser Alan J. 
Published: April 2004

AbstractThe origins of elasticity in thermoplastic vulcanizates have been debated for the past decade. Previous modeling attempts provide numerical solutions that make assessment of constituent concentration and interaction unclear. A microcellular modeling approach is proposed and evaluated herein to describe the steady‐state behavior of dynamically vulcanized blends of ethylene‐propylene‐diene monomer (EPDM) and isotactic polypropylene (iPP). This approach provides an analytic result including terms for composition and cure state. Three types of deformation are accounted for: elastic and plastic deformation of iPP, elastic deformation of EPDM, and localized elastic and plastic rotation about iPP junction points. The viability of the constitutive model is evaluated in terms of iPP concentration and EPDM cure state.