bluuebunny/crossref_metadata_2025_split huggingface dataset:
Movement disorders in musicians
Authors: Jankovic Joseph , Ashoori Aidin 
Published: September 2008

AbstractThe focus of this article is to review the epidemiology, phenomenology, pathophysiology, genetics, and treatment of movement disorders, particularly task‐specific dystonia, in musicians. The goal is to draw attention to this group of neurological disorders among musicians, music teachers, and healthcare professionals and to highlight the importance of early diagnosis, therapeutic options, and preventive measures. To increase professional and public awareness and to facilitate the recognition of music‐related neurological problems, we suggest that “medical problems of musicians” be included in curriculum of music schools and medical schools. © 2008 Movement Disorder Society
Consanguineous Iranian kindreds with severe Tourette syndrome
Authors: Motlagh Maria G. , Seddigh Arshia , Dashti Behnoosh , Leckman James F. , Alaghband‐Rad Javad 
Published: September 2008

AbstractThe search for vulnerability genes for Tourette syndrome has been ongoing for nearly three decades. The contribution of recessive loci with reduced penetrance is one possibility that has been difficult to explore. Homozygosity mapping has been successfully used to detect recessive loci within populations with high rates of consanguinity. Using this technique, even quite small inbred families can be informative due to autozygosity in which the two alleles at an autosomal locus are identical by descent (i.e., copies of a single ancestral gene). To explore the utility of this approach, we identified 12 consanguineous Iranian families. Remarkably, these families were seen with an unusual natural history characterized by the early onset of vocal tics and coprolalia and frequent comorbidity with obsessive‐compulsive disorder. Genotyping the affected and unaffected members of these pedigrees has the potential to identify rare recessive contributions to this disorder. © 2008 Movement Disorder Society
Cost‐effectiveness of123I‐FP‐CIT SPECT in the differential diagnosis of essential tremor and Parkinson's disease in Italy
Authors: Antonini Angelo , Berto Patrizia , Lopatriello Stefania , Tamma Filippo , Annemans Lieven , Chambers Mike 
Published: September 2008

AbstractEconomic evaluation (Italian NHS perspective) modeling123I‐FP‐CIT SPECT (DaTSCAN®) compared to clinical judgment alone for differentiating essential tremor (ET) from Parkinson's Disease (PD). A 5‐year Markov model was constructed to assess the cost‐effectiveness of123I‐FP‐CIT SPECT to differentiate ET from PD in patients referred to a movement disorder specialist in Italy. Published data and a double‐round, Delphi panel of 12 specialists populated the model. Effectiveness was expressed as the projected Years on potentially beneficial therapy (PBTYs). Costs were expressed in Euros (2005 values). The model suggests that over 5 years, the “current” diagnostic pathway generated an average of 2.3 PBTYs/patient at an estimated cost of €8,864.123I‐FP‐CIT SPECT generated an average of 4.1 PBTYs/patient at an estimated cost of €8,422, which represented an additional 1.8 PBTYs at a cost saving of €442/patient (€341 when discounted at 5%). The estimated cost‐effectiveness of123I‐FP‐CIT SPECT is under €1,000 per PBTY gained when the underlying disease prevalence is high (55–70%), and cost‐saving at prevalence under 55%.123I‐FP‐CIT SPECT is likely to be regarded as economically advantageous to differentiate ET from PD, increasing time on potentially beneficial therapy at a lower overall cost to the healthcare system. © 2008 Movement Disorder Society
The PSP‐associated MAPT H1 subhaplotype in Guadeloupean atypical Parkinsonism
Authors: Camuzat Agnès , Romana Marc , Dürr Alexandra , Feingold Josué , Brice Alexis , Ruberg Merle , Lannuzel Annie 
Published: September 2008

AbstractThe aim of this study was to determine whether the H1 subhaplotype in MAPT associated with progressive supranuclear palsy (PSP) in Caucasians confers risk for PSP‐like atypical parkinsonism in Guadeloupe, a tauopathy. Guadeloupean controls and patients with atypical and idiopathic parkinsonism and ethnically and age‐matched controls were genotyped for H1 and H2 alleles, then for the H1 subhaplotype associated with PSP in Caucasians, using previously described haplotype‐tagging single nucleotide polymorphisms (Ht‐SNPs) in linkage disequilibrium at the MAPT locus. Most Guadeloupean controls and patients were homozygous for the H1 allele; only 5% were heterozygous for the H2 allele, consistent with the European contribution to the racial admixture in Guadeloupe, but equivalent to the frequency found in Caucasian PSP patients. The frequencies of the Ht‐SNPs used to determine the PSP‐associated H1 subhaplotype in both Guadeloupean controls and parkinsonians were similar, indicating that the H1 subhaplotype associated with PSP in Caucasians was not a risk factor for PSP‐like atypical parkinsonism in Guadeloupe. Interestingly, they were also similar to the frequencies in Caucasian PSP patients. The major H1 subhaplotype in Guadeloupe, determined by analysis of linkage desequibrium, differed from the major Caucasian subhaplotype, but corresponded to minor alleles previously described. © 2008 Movement Disorder Society
NMR and theoretical study of the cooperative interaction of hydrated proton with dibenzo‐24‐crown‐8
Authors: Kříž Jaroslav , Dybal Jiří , Budka Jan , Makrlík Emanuel 
Published: September 2008

AbstractInteraction of hydrated protons (HPs) with dibenzo‐24‐crown‐8 (DBC in nitrobenzene‐d5 was studied by 1H and 13C NMR under assistance of ab initio‐density functional theory (DFT) quantum calculations. HPs were afforded by hydrogen bis(1,2‐dicarbollyl) cobaltate (HDCC) with 3.5 M excess of H2O. The forming of a complex between HP and DBC leads to marked and additive relative shifts of both 1H and 13C signals. This was utilized for the estimation of the stabilization constant K of the complex. Its value is at least 106 l/mol, which agrees with the result of independent extraction method (logK = 6.3). Using absolute integral intensities of the HP signal in a water‐saturated system, it was shown that the form of HP present in the complex must be H5O2+, in accord with formerly published structure of the complex in crystalline form. The investigation of the dynamics of exchange between bound and free DBC by transverse relaxation using variably spaced pulses in the Carr–Purcell–Meiboom–Gill (CPMG) sequence or on‐resonance rotating‐frame relaxation with variable spin‐lock field intensity was partly hampered by the fast relaxation of some signals in the complex because of relative immobilization of its internal motions. In order to remove these effects, a pulse sequence dipolar interaction‐free transverse relaxation (DIFTRE) for static DIFTRE was devised and the MLEV17 pulse sequence with high intensity of electromagnetic field was used in a separate series of experiments. Using the results of these latter experiments, the correlation time of exchange was established to be about 0.8 ms, which complied with the shape of the spectra. The accompanying ab initio DFT calculations showed that the apparent symmetry of the molecules of both DBC and its complex with H5O2+ was probably the result of averaging of energetically close conformations (five for DBC and four for the complex). Both NMR and the calculations show that the basic mode of binding of the ion in the complex is analogous to that found in crystal but the most pronounced conformation is slightly different. Copyright © 2008 John Wiley & Sons, Ltd.
Epithelial stem cells in corneal regeneration and epidermal gene therapy
Authors: Pellegrini G , Rama P , Mavilio F , De Luca M 
Published: September 2008

AbstractRegenerative medicine refers to innovative therapies aimed at the permanent restoration of diseased tissues and organs. Regeneration of self‐renewing tissues requires specific adult stem cells, which need to be genetically modified to correct inherited genetic diseases. Cultures of epithelial stem cells permanently restore severe skin and mucosal defects, and genetically corrected epidermal stem cells regenerate a normal epidermis in patients carrying junctional epidermolysis bullosa. The keratinocyte stem cell is therefore the only cultured stem cell used both in cell therapy and gene therapy clinical protocols. Epithelial stem cell identification, fate and molecular phenotype have been extensively reviewed, but not in relation to tissue regeneration. In this paper we focus on the localization and molecular characterization of human limbal stem cells in relation to corneal regeneration, and the gene therapy of genetic skin diseases by means of genetically modified epidermal stem cells. Copyright © 2008 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
TTF1 expression in non‐small cell lung carcinoma: association with TTF1 gene amplification and improved survival
Authors: Perner S , Wagner PL , Soltermann A , LaFargue C , Tischler V , Weir BA , Weder W , Meyerson M , Giordano TJ , Moch H , Rubin MA 
Published: September 2008

AbstractAcquired chromosomal aberrations play an important role in tumour development and progression. Such genetic alterations occur in a significant proportion of non‐small cell lung carcinomas (NSCLCs) and include amplification of 14q13.3, which contains the TTF1 gene. We asked whether TTF1 amplification is associated with increased TTF1 protein expression in NSCLCs, and whether TTF1 is associated with clinicopathological features, including patient survival. We used a FISH assay and quantitative immunohistochemical staining to interrogate a population‐based cohort of 538 NSCLCs from Swiss patients for TTF1 amplification and protein expression. We found TTF1 amplification in ∼13% of adenocarcinomas (ACs) and in ∼9% of squamous cell carcinomas (SCCs) and TTF1 amplification was associated with increased TTF1 protein expression. High‐level TTF1 expression was significantly associated with smaller tumour size, female gender and longer overall survival only among ACs (median survival 82 versus 28 months; p = 0.002). On multivariate analysis, high TTF1 expression was an independent predictor of favourable prognosis in patients with AC [hazard ratio, 0.56 (95% CI 0.38–0.83); p = 0.008]. We conclude that TTF1 amplification is a mechanism of high‐level TTF1 expression in a subset of NSCLCs. When expressed at high levels, this routinely used diagnostic marker is also an independent biomarker of favourable prognosis in AC. Copyright © 2008 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
5α‐Dihydrotestosterone (DHT) retards wound closure by inhibiting re‐epithelialization
Authors: Gilliver SC , Ruckshanthi JPD , Hardman MJ , Zeef LAH , Ashcroft GS 
Published: September 2008

AbstractThe ongoing search for explanations as to why elderly males heal acute skin wounds more slowly than do their female counterparts (and are more strongly disposed to conditions of chronic ulceration) has identified endogenous oestrogens and androgens as being respectively enhancers and inhibitors of repair. We previously demonstrated that blocking the conversion of testosterone to 5α‐dihydrotestosterone (DHT) limits its ability to impair healing, suggesting that DHT is a more potent inhibitor of repair than is testosterone. The present study aimed to delineate the central mechanisms by which androgens delay repair. Whilst the contractile properties of neither rat woundsin vivonor fibroblast‐impregnated collagenous discsin vitroappeared to be influenced by androgen manipulations, the global blockade of DHT biosynthesis markedly accelerated re‐epithelialization of incisional and excisional wounds and reduced local expression of β‐catenin, a key inhibitor of repair. Moreover, DHT retarded thein vitromigration of epidermal keratinocytes following scratch wounding. By contrast, it failed to influence the migratory and proliferative properties of dermal fibroblasts, suggesting that its primary inhibitory effect is upon re‐epithelialization. These novel findings may be of particular significance in the context of chronic ulceration, for which being male is a key risk factor. Copyright © 2008 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
The EWSR1/NR4A3 fusion protein of extraskeletal myxoid chondrosarcoma activates the PPARG nuclear receptor gene
Authors: Filion C , Motoi T , Olshen AB , Laé M , Emnett RJ , Gutmann DH , Perry A , Ladanyi M , Labelle Y 
Published: September 2008

AbstractThe NR4A3 nuclear receptor is implicated in the development of extraskeletal myxoid chondrosarcoma (EMC), primitive sarcoma unrelated to conventional chondrosarcomas, through a specific fusion with EWSR1 resulting in an aberrant fusion protein that is thought to disrupt the transcriptional regulation of specific target genes. We performed an expression microarray analysis of EMC tumours expressing the EWSR1/NR4A3 fusion protein, comparing their expression profiles to those of other sarcoma types. We thereby identified a set of genes significantly overexpressed in EMC relative to other sarcomas, including PPARG and NDRG2. Western blot or immunohistochemical analyses confirm that PPARG and NDRG2 are expressed in tumours positive for EWSR1/NR4A3. Bioinformatic analysis identified a DNA response element for EWSR1/NR4A3 in the PPARG promoter, and band‐shift experiments and transient transfections indicate that EWSR1/NR4A3 can activate transcription through this element. Western blots further show that an isoform of the native NR4A3 receptor lacking the C‐terminal domain is very highly expressed in tumours positive for EWSR1/NR4A3, and co‐transfections of this isoform along with EWSR1/NR4A3 indicate that it may negatively regulate the activity of the fusion protein on the PPARG promoter. These results suggest that the overall expression of PPARG in EMC may be regulated in part by the balance between EWSR1/NR4A3 and NR4A3, and that PPARG may play a crucial role in the development of these tumours. The specific up‐regulation of PPARG by EWSR1/NR4A3 may also have potential therapeutic implications. Copyright © 2008 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
A new role for the neuronal ubiquitin C‐terminal hydrolase‐L1 (UCH‐L1) in podocyte process formation and podocyte injury in human glomerulopathies
Authors: Meyer‐Schwesinger C , Meyer TN , Münster S , Klug P , Saleem M , Helmchen U , Stahl RAK 
Published: September 2008

AbstractGlomerular epithelial cell (podocyte) injury is characterized by foot process retraction, slit diaphragm reorganization, and degradation of podocyte‐specific proteins. However, the mechanisms underlying podocyte injury are largely unknown. The ubiquitin C‐terminal hydrolase‐L1 (UCH‐L1) is a key modulator of ubiquitin modification in neurons. Like neurons, UCH‐L1 expression was associated with an undifferentiated status in cultured human podocytes, whereas differentiation and arborization decreased UCH‐L1 and monoUb expression. Inhibition of UCH‐L1 induced time and concentration‐dependent process formation with α‐actinin‐4 distribution to the cell membrane and processes. An immunohistochemical approach was used to evaluate whether UCH‐L1 expression was associated with podocyte injury in 15 different human glomerular diseases. Whereas normal kidneys expressed no UCH‐L1 and little ubiquitin, a subset of human glomerulopathies associated with podocyte foot process effacement (membranous nephropathy, SLE class V, FSGS) de novo expressed UCH‐L1 in podocyte cell bodies, nuclei, and processes. Interestingly, UCH‐L1 expression correlated with podocyte ubiquitin content and internalization of the podocyte‐specific proteins nephrin and α‐actinin‐4. In contrast, minimal change glomerulonephritis, a reversible disease, demonstrated minimal UCH‐L1 and ubiquitin expression with intact α‐actinin‐4 but internalized nephrin. Glomerular kidney diseases typically not associated with foot process effacement (SLE class IV, ANCA+ necrotizing GN, amyloidosis, IgA nephritis) expressed intermediate to no UCH‐L1 and ubiquitin. These studies show a role for UCH‐L1 and ubiquitin modification in podocyte differentiation and injury. Copyright © 2008 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Preparation and friction properties of PBT/MMT composites
Authors: Mu Bo , Wang Qihua , Wang Tingmei , Wang Honggang , Jian Lingqi , Pei Xianqiang 
Published: September 2008

AbstractPoly(butylene terephthalate)/clay composites were prepared by direct melt compounding. Two structures were formed as confirmed by XRD and transmission electron microscopy. The presence of MMT layers does not change the crystal structure and the degree of crystallinity of PBT matrix. The tribological behaviors of neat PBT and PBT/clay composites were studied by the means of a pin‐on‐disk apparatus. It was found that the intercalation of organoclay could help reduce the friction coefficient and the specific wear rate of PBT, while the addition of natural clay was harmful to the friction coefficient and wear resistance of the polymer matrix. The different tribological behaviors of the composites are due to their different morphologies and the difference in the interfacial adhesion between the matrix and the clay platelets. POLYM. COMPOS., 2009. © 2008 Society of Plastics Engineers
Promoting the adhesion of high‐performance polymer fibers using functional plasma polymer coatings
Authors: Sugihara H. , Jones F.R. 
Published: September 2008

AbstractPlasma‐copolymerized functional coatings of acrylic acid and 1,7‐octadiene were deposited onto high strength, high modulus, poly‐p‐phenylene benzobisoxazole (PBO) fibers. X‐ray photoelectron spectroscopy (XPS) with trifluoroethanol derivatization confirmed that the PBO fibers were covered completely with the plasma copolymer and that the coating contained a quantitative concentration of carboxylic acid groups. Microdebond single filament adhesion and interlaminar shear strength (ILSS) tests were used to evaluate the interfacial strength of epoxy resin composites containing these functionalized PBO fibers. Both the interfacial shear strength (IFSS) obtained from single filament tests, and the ILSS of high volume fraction composites were a function of the surface functionality of the fibers so that there was a good correlation between ILSS and IFSS data. The tensile strengths of single fibers with or without coating were comparable, demonstrating that the fiber surface was not damaged in the plasma‐coating procedure. Indeed, the statistical analysis showed that Weibull modulus was increased. Therefore, plasma‐polymerized coatings can be used to control the interfacial bond between PBO fibers and matrix resins and act as a protective size for preserving the mechanical properties of the fibers. POLYM. COMPOS., 2009. © 2008 Society of Plastics Engineers
Use of different alkylammonium salts in clay surface modification for epoxy‐based nanocomposites
Authors: Nakas G. Ipek , Kaynak Cevdet 
Published: September 2008

AbstractLayered silicates become widely used reinforcement material in the polymer nanocomposite production in recent years due to their high aspect ratio, ease of processing, and low cost. In this present study, the aim was to evaluate the usability of a raw clay source (Resadiye, Turkey) in the production of epoxy/clay nanocomposites and to investigate the effects of different surface modifiers. For this purpose, raw Na–montmorillonite clay was first purified and then surface modified by using different types of alkylammonium salts: tetramethyl ammonium bromide, benzyl triethyl ammonium bromide, dodecyl trimethyl ammonium bromide, hexadecyl trimethyl ammonium bromide, and octadecyl trimethyl ammonium bromide. Purification and surface modification of this clay were evaluated by using the following analyses; X‐ray diffraction (XRD), cation exchange capacity (CEC), particle size distribution, and dissolved organic content. These analyses simply indicated that surface modification increased both interlayer spacing between the silicate layers and CEC of the clay. These improvements were directly proportional with the chain length of the surface modifier. Nanocomposite specimens were produced by adding 0.5 wt% surface modified clay into the epoxy matrix. These specimens were characterized by XRD, Si‐mapping facility of SEM, and mechanical tests. XRD results indicated an exfoliated structure whereas Si‐mapping showed the uniform distribution of clay particles in epoxy, leading to improved mechanical properties, for instance more than 100% increase in fracture toughness of the neat epoxy specimen. POLYM. COMPOS., 2009. © 2008 Society of Plastics Engineers
Preparation and properties of nanoparticle and long‐fiber‐reinforced unsaturated polyester composites
Authors: Zhou Gang , Movva Siva , Lee L. James 
Published: September 2008

AbstractIn this study, a new approach was used to prepare polymer composites reinforced by both nanoparticles and continuous fibers. Carbon nanofibers were prebound onto glass fiber mats, and then unsaturated polyester composites were prepared by vacuum‐assisted resin transfer molding. Mechanical and thermal properties of these composites were measured and compared with those of the composites synthesized by premixing carbon nanofibers with the polymer resin. Flexural strength and modulus of composites improved with the incorporation of nanoparticles. Specifically, the property improvement was higher in the case of the composites prepared by the new prebound method. It was also found that carbon nanofibers increased the glass transition temperature and reduced the thermal expansion coefficient of unsaturated polyester composites. POLYM. COMPOS., 2009. © 2008 Society of Plastics Engineers
Epoxy‐ and polyester‐based composites reinforced with glass, carbon and aramid fabrics: Measurement of heat capacity and thermal conductivity of composites by differential scanning calorimetry
Authors: Cecen Volkan , Tavman Ismail H. , Kok Mediha , Aydogdu Yildirim 
Published: September 2008

AbstractThe primary purpose of the study is to investigate the temperature dependence of heat capacity and thermal conductivity of composites having different fiber/matrix combinations by means of heat‐flux differential scanning calorimetry (DSC). The materials used as samples in this study were epoxy‐ and polyester‐based composites. Noncrimp stitched glass, carbon, and aramid fabric were used as reinforcements for making unidirectional composites. For the heat capacity measurements the composite sample and a standard material are separately subjected to same linear temperature program. By recording the heat flow rate into the composite sample as a function of temperature, and comparing it with the heat flow rate into a standard material under the same conditions, the temperature dependence of heat capacity of the composite sample is determined. Measurements were carried out over a wide range of temperatures from about 20 to 250°C. The differential scanning calorimeter was adapted to perform the thermal conductivity measurements in the direction perpendicular to the fiber axis over the temperature range of 45–235°C. The method used in this study utilizes the measurement of rate of heat flow into a sensor material during its first‐order phase transition to obtain the thermal resistance of a composite material placed between the sensor material and the heater in the DSC. POLYM. COMPOS., 2009. © 2008 Society of Plastics Engineers