bluuebunny/crossref_metadata_2025_split huggingface dataset:
On the Origin of the Transthyretin Val30Met Familial Amyloid Polyneuropathy
Authors: Zaros C. , Genin E. , Hellman U. , Saporta M. A. , Languille L. , Wadington‐Cruz M. , Suhr O. , Misrahi M. , Planté‐Bordeneuve V. 
Published: May 2008

SummaryTransthyretin (TTR) familial amyloid polyneuropathy is a severe autosomal dominant neuropathy of adulthood, frequently linked to the pathogenic Val30Met variant of the TTR gene. The condition was initially described in northern Portugal, which is the first focus of the disease. Other important clusters of families are found in Sweden, Japan and South America.The origin of the Val30Met mutation and its distribution through the populations remains unclear. In the present work, we aimed at refining the history of the Val30Met mutation in patients affected with TTR amyloid neuropathy from Portugal, Sweden and Brazil. The decay of haplotype sharing was studied in 60 patients to estimate the age of the Most Recent Common Ancestor (MRCA) of mutation carriers in these populations. Our results showed a common haplotype in Portuguese and Brazilian patients and an age estimate of the MRCA of 750 and 650 years, respectively. In contrast, a different haplotype was found in the Swedish Val30Met patients with a corresponding age estimate for the MRCA, of 375 years.This work strengthens the hypothesis of different founders in Portuguese and Swedish Val30Met carriers and suggested a Portuguese origin of the Brazilian mutation. The age estimates of the MRCA are in line with the current historical knowledge of these populations.
The impact of ethical concerns on family consumer decision‐making
Authors: Carey Lindsey , Shaw Deirdre , Shiu Edward 
Published: May 2008

AbstractEthical consumerism and family consumer decision‐making, including the influence of children in this area, are spheres of consumer behaviour in which a substantial amount of academic research has already been undertaken. However, the crossover of these two areas is as yet under‐researched, as well as the level of pester power parents are subjected to from children aged 3 and under. This paper uses qualitative methods to investigate the issues surrounding the ethical consumer decision‐making process with families who have children aged 3 years old or under. This research found that the motivation to pursue an ethical lifestyle varied across the sample, but the emergence of an ‘inheritance factor’, where parents are awakened to ethical issues because of the birth of their child, was prominent. Other issues that transpired from this research include the prominence of ethical trade‐offs in consumer decision‐making, ethical choices as normalizing behaviour and finally the presence of pester power in the ethical context. Ethical consumerism strongly emerged as an integral part of the parent's identity construction, especially for the mothers as they struggled to adapt to their new roles. Further research delving into the role ethical choices have on identity construction and the parental response to pester power would be a valuable addition to the overall context of this research.
Consumer product search and purchase behaviour using various retail channels: the role of perceived retail usefulness
Authors: Kim Jihyun , Lee Hyun‐Hwa 
Published: May 2008

AbstractThe purpose of this study was to examine the influences of consumers' perceptions of retail usefulness for product information search and their previous purchase satisfaction on their frequencies of product information search and product purchase behaviours for apparel products. These relationships were investigated in five retail settings – Internet shopping, catalogue shopping, television shopping, local retail shopping, and non‐local retail shopping. One hundred seventy‐six students in a US Midwestern university provided usable responses. The results of causal model analyses showed that the proposed model fits the data well for all five retail channels. Consumers who perceived a certain retail channel more useful for product information search searched for product information more frequently via that retail channel, and purchased products more often via that retail channel. Consumers who were more satisfied with apparel purchases from a retail channel purchased the products more frequently via that retail channel. Theoretical and managerial implications are discussed.
Environmental value chain management of laundry detergents in the use phase
Authors: Paloviita Ari , Järvi Pentti 
Published: May 2008

AbstractEnvironmental value chain management is concerned with the environmental aspects of products and services along the whole value chain, from raw material and energy acquisition to waste management. The use phase in the value chain remains an understudied subject, despite considerable consumption‐related environmental impacts. The purpose of this paper is to analyse the use phase of laundry detergents in households. Our empirical data consist of interviews in the laundry rooms of 299 Finnish households. Consumers’ environmental awareness, use of dosage measuring devices and actual doses of laundry detergents are studied in the empirical part of the study. The results show that consumers do not perceive households as significant polluters, the measuring devices they use are diverse and the number of ‘heavy users’ is high in all detergent brands. It is concluded that in the consumption of laundry detergents, consumers do not perceive environmental issues as specific value criteria in relation to the use of laundry detergents. Manufacturers, marketers and other actors in the value chain of laundry detergents could contribute to alleviating the harmful environmental impacts of the use phase by more effective consumer education and better product design. For consumers, it might be beneficial to adopt eco‐efficiency thinking in their daily consumption.
Depletion of 14‐3‐3 zeta elicits endoplasmic reticulum stress and cell death, and increases vulnerability to kainate‐induced injury in mouse hippocampal cultures
Authors: Murphy Niamh , Bonner Helena P. , Ward Manus W. , Murphy Brona M. , Prehn Jochen H. M. , Henshall David C. 
Published: May 2008

Abstract14‐3‐3 proteins are ubiquitous signalling molecules that regulate development and survival pathways in brain. Altered expression and cellular localization of 14‐3‐3 proteins has been implicated in neurodegenerative diseases and in neuronal death after acute neurological insults, including seizures. Presently, we examined expression and function of 14‐3‐3 isoforms in vitro using mouse organotypic hippocampal cultures. Treatment of cultures with the endoplasmic reticulum (ER) stressor tunicamycin caused an increase in levels of 14‐3‐3 zeta within the ER‐containing microsomal fraction, along with up‐regulation of Lys‐Asp‐Glu‐Leu‐containing proteins and calnexin, and the selective death of dentate granule cells. Depletion of 14‐3‐3 zeta levels using small interfering RNA induced both ER stress proteins and death of granule cells. Treatment of hippocampal cultures with the excitotoxin kainic acid increased levels of Lys‐Asp‐Glu‐Leu‐containing proteins and microsomal 14‐3‐3 zeta levels and caused cell death within the CA1, CA3 and dentate gyrus of the hippocampus. Kainic acid‐induced damage was significantly increased in each hippocampal subfield of cultures treated with small interfering RNA targeting 14‐3‐3 zeta. The present data indicate a role for 14‐3‐3 zeta in survival responses following ER stress and possibly protection against seizure injury to the hippocampus.
Kv 1.1 is associated with neuronal apoptosis and modulated by protein kinase C in the rat cerebellar granule cell
Authors: Hu Chang‐Long , Zeng Xi‐Min , Zhou Meng‐Hua , Shi Ying‐Tang , Cao Hong , Mei Yan‐Ai 
Published: May 2008

AbstractPreviously, we reported that apoptosis of cerebellar granular neurons induced by low‐K+ and serum‐free (LK‐S) was associated with an increase in the A‐type K+ channel current (IA), and an elevated expression of main α‐subunit of the IA channel, which is known as Kv4.2 and Kv4.3. Here, we show, as assessed by quantitative RT‐PCR and whole‐cell recording, that besides Kv4.2 and Kv4.3, Kv1.1 is very important for IA channel. The expression of Kv1.1 was elevated in the apoptotic neurons, while silencing Kv1.1 expression by siRNA reduced the IA amplitude of the apoptotic neuron, and increased neuron viability. Inhibiting Kv1.1 current by dendrotoxin‐K evoked a similar effect of reduction of IA amplitude and protection of neurons. Applying a protein kinase C (PKC) activator, phorbol ester acetate A (PMA) mimicked the LK‐S‐induced neuronal apoptotic effect, enhanced the IA amplitude and reduced the granule cell viability. The PKC inhibitor, bisindolylmaleimide I and Gö6976 protected the cell against apoptosis induced by LK‐S. After silencing the Kv1.1 gene, the effect of PMA on the residual K+ current was reduced significantly. Quantitative RT‐PCR and Western immunoblot techniques revealed that LK‐S treatment and PMA increased the level of the expression of Kv1.1, in contrast, bisindolylmaleimide I inhibited Kv1.1 expression. In addition, the activation of the PKC isoform was identified in apoptotic neurons. We thus conclude that in the rat cerebellar granule cell, the IA channel associated with apoptotic neurons is encoded mainly by the Kv1.1 gene, and that the PKC pathway promotes neuronal apoptosis by a brief modulation of the IA amplitude and a permanent increase in the levels of expression of the Kv1.1 α‐subunit.
Biochemical characterization of membrane‐associated septin from rat brain
Authors: Maimaitiyiming Maowulan , Kumanogoh Haruko , Nakamura Shun , Nagata Koh‐Ichi , Suzaki Toshinobu , Maekawa Shohei 
Published: May 2008

AbstractWithin the cell membrane there exist various microdomains (lipid rafts) in which specific lipids and proteins are assembled and these microdomains are recovered in the detergent‐resistant low‐density membrane fraction (DRM). Septin is a novel GTP‐binding, cytoskeletal protein having various isoforms that assemble into homo‐ and heterooligomers and filaments. As the localization of septin 3 in DRM was found through a proteomics analysis of brain‐derived DRM, the presence of other septin isoforms in DRM was studied. Western blotting analysis showed maturation‐dependent enrichment of several septin isoforms in DRM prepared from synaptic plasma membrane (SPM). These isoforms were solubilized with high MgCl2 solution and recovered as the precipitate after dialysis to low ionic solution. Three times cycling of the extraction‐dialysis process resulted in the partial purification of septin complex and electron microscopic observation of this fraction revealed rod‐like structures in which building units were observed. The presence of heterooligomers was shown with western blotting after the separation of the MgCl2 extract with blue‐native polyacrylamide gel electrophoresis. Immunoprecipitation assay using monoclonal anti‐septin11 antibody also showed the presence of heterooligomers. These results show that septin localizes in the membrane microdomains of the SPM in adult brain and may have important roles in the membrane dynamics of neurons.
Functions of human bitter taste receptors depend on N‐glycosylation
Authors: Reichling Claudia , Meyerhof Wolfgang , Behrens Maik 
Published: May 2008

AbstractHuman bitter taste receptors of the TAS2R gene family play a crucial role as warning sensors against the ingestion of toxic food compounds. Moreover, the genetically highly polymorphic hTAS2Rs recognize an enormous number of structurally diverse toxic and non‐toxic bitter substances, and hence, may substantially influence our individual eating habits. Heterologous expression in mammalian cells is a useful tool to investigate interactions between these receptors and their agonists. However, many bitter taste receptors are poorly expressed at the cell surface of heterologous cells requiring the addition of plasma membrane export promoting epitopes to the native receptor proteins. Currently, nothing is known about amino acid motifs or other receptor‐intrinsic features of TAS2Rs affecting plasma membrane association. In the present study, we analyzed the Asn‐linked glycosylation of hTAS2Rs at a consensus sequence in the second extracellular loop, which is conserved among all 25 hTAS2Rs. Non‐glycosylated receptors exhibit substantially lower cell surface localization and reduced association with the cellular chaperone calnexin. As the auxiliary factors receptor transporting proteins 3 and 4 are able to restore the function of non‐glycosylated hTAS2R16 partially, we conclude that glycosylation is important for receptor maturation but not for its function per se.
Metabolic syndrome and the role of dietary lifestyles in Alzheimer’s disease
Authors: Pasinetti Giulio Maria , Eberstein Jacqueline A. 
Published: May 2008

AbstractSince Alzheimer’s disease (AD) has no cure or preventive treatment, an urgent need exists to find a means of preventing, delaying the onset, or reversing the course of the disease. Clinical and epidemiological evidence suggests that lifestyle factors, especially nutrition, may be crucial in controlling AD. Unhealthy lifestyle choices lead to an increasing incidence of obesity, dyslipidemia and hypertension – components of the metabolic syndrome. These disorders can also be linked to AD. Recent research supports the hypothesis that calorie intake, among other non‐genetic factors, can influence the risk of clinical dementia. In animal studies, high calorie intake in the form of saturated fat promoted AD‐type amyloidosis, while calorie restriction via reduced carbohydrate intake prevented it. Pending further study, it is prudent to recommend to those at risk for AD – e.g. with a family history or features of metabolic syndrome, such as obesity, insulin insensitivity, etc. – to avoid foods and beverages with added sugars; to eat whole, unrefined foods with natural fats, especially fish, nuts and seeds, olives and olive oil; and to minimize foods that disrupt insulin and blood sugar balance.
Role of AMPA receptor trafficking in NMDA receptor‐dependent synaptic plasticity in the rat lateral amygdala
Authors: Yu Shu Yan , Wu Dong Chuan , Liu Lidong , Ge Yuan , Wang Yu Tian 
Published: May 2008

AbstractStimulated exocytosis and endocytosis of post‐synaptic α‐amino‐3‐hydroxy‐5‐methyl‐4‐isoxazolepropionic acid subtype of glutamate receptors (AMPARs) have been proposed as primary mechanisms for the expression of hippocampal CA1 long‐term potentiation (LTP) and long‐term depression (LTD), respectively. LTP and LTD, the two most well characterized forms of synaptic plasticity, are thought to be important for learning and memory in behaving animals. Both LTP and LTD can also be induced in the lateral amygdala (LA), a critical structure involved in fear conditioning. However, the role of AMPAR trafficking in the expression of either LTP or LTD in this structure remains unclear. In this study, we show that NMDA receptor‐dependent LTP and LTD can be reliably induced at the synapses of the auditory thalamic inputs to the LA in brain slices. The expression of LTP was prevented by post‐synaptic blockade of vesicle‐mediated exocytosis with application of a light chain of Clostridium tetanus neurotoxin and was associated with increased cell‐surface AMPAR expression. In contrast, the expression of LTD was prevented by post‐synaptic application of a glutamate receptor 2‐derived interference peptide, which specifically blocks the stimulated clathrin‐dependent endocytosis of AMPARs, and was correlated with a reduction in plasma membrane‐surface expression of AMPARs. These results strongly suggest that regulated trafficking of post‐synaptic AMPARs is also involved in the expression of LTP and LTD in the LA.
Zebrafish as a new animal model for movement disorders
Authors: Flinn Laura , Bretaud Sandrine , Lo Christine , Ingham Phillip W. , Bandmann Oliver 
Published: May 2008

AbstractThe zebrafish, long recognized as a model organism for the analysis of basic developmental processes, is now also emerging as an alternative animal model for human diseases. This review will first provide an overview of the particular characteristics of zebrafish in general and their dopaminergic nervous system in particular. We will then summarize all work undertaken so far to establish zebrafish as a new animal model for movement disorders and will finally emphasize its particular strength – amenability to high throughput in vivo drug screening.
Regulation of DYT1 gene expression by the Ets family of transcription factors
Authors: Armata Ioanna A. , Ananthanarayanan Meenakshisundaram , Balasubramaniyan Natarajan , Shashidharan Pullanipally 
Published: May 2008

AbstractThe DYT1 gene encodes for torsinA, a protein with widespread tissue distribution, involved in early onset dystonia (EOD). Numerous studies have focused on torsinA function but no information is available on its transcriptional regulation. We cloned mouse and human 5′‐upstream DYT1 DNA fragments, exhibiting high transcriptional activity, as well as tissue specificity. We identified a proximal minimal DYT1 promoter within −141 bp for mouse and −191 bp for human with respect to the ATG codon. Primer extension analysis indicated multiple transcription start sites. In silico analysis of approximately 500 bp 5′‐upstream DYT1 fragment demonstrated lack of a classical TATA or CAAT box and the presence of a highly conserved direct repeat of two Ets binding cores within −86 bp to −77 bp and −78 bp to −69 bp of the mouse and human DYT1 gene, respectively. A single or a two base nucleotide alteration within the downstream Ets core resulted in approximately 90% (mouse) or 45–60% (human) drop in activity. Interestingly, a 3‐bp distance increase between the two Ets cores dramatically decreased transcriptional activity which was partially restored when the distance was increased up to 10 bp. Ets‐like dominant negatives confirmed the Ets factors as DYT1 transcriptional activators.
Telomeres and race: what can we learn about human biology from health differentials?
Authors: Kaufman Jay S. , Cooper Richard S. 
Published: May 2008

SummaryThe advent of molecular technology that can be applied across large population samples has added – rather than reduced – complexity in the analysis of the intertwined effects of social history and heritable factors on health outcomes. The report by Hunt et al. in this issue of Aging Cell provides an example of the promises and dilemmas associated with this increased complexity.
Leukocyte telomeres are longer in African Americans than in whites: the National Heart, Lung, and Blood Institute Family Heart Study and the Bogalusa Heart Study
Authors: Hunt Steven C. , Chen Wei , Gardner Jeffrey P. , Kimura Masayuki , Srinivasan Sathanur R. , Eckfeldt John H. , Berenson Gerald S. , Aviv Abraham 
Published: May 2008

SummaryLeukocyte telomere length (LTL) is ostensibly a bio‐indicator of human aging. Here we report that African Americans have longer LTL than whites. We studied cross‐sectionally 2453 individuals from the National Heart, Lung, and Blood Institute (NHLBI) Family Heart Study (age = 30–93 years) and the Bogalusa Heart Study (age = 19–37 years), comprising 1742 whites and 711 African Americans. We measured LTL by Southern blots of the terminal restriction fragments length. In 234 participants, telomere repeats were also measured by quantitative polymerase chain reaction (qPCR). Adjusted for age and body mass index (BMI), the respective leukocyte telomere lengths (mean ± SEM) were considerably longer in African Americans than in whites both in the Family Heart Study (7.004 ± 0.033 kb vs. 6.735 ± 0.024 kb, p < 0.0001) and the Bogalusa Heart Study (7.923 ± 0.063 kb vs. 7.296 ± 0.039 kb, p < 0.0001). We confirmed the racial effect on LTL by qPCR (3.038 ± 0.565 T/S units for African Americans vs. 2.714 ± 0.487 T/S units for whites, p < 0.001). Cross‐sectionally, sex‐ and BMI‐adjusted LTL became shorter with age (range 19–93 years) at a steeper slope in African Americans than in whites (0.029 kb year−1 vs. 0.020 kb year−1, respectively, p = 0.0001). We suggest that racial difference in LTL arises from a host of interacting biological factors, including replication rates of hematopoietic stem cells.
How Many Nurses per Patient? Measurements of Nurse Staffing in Health Services Research
Authors: Spetz Joanne , Donaldson Nancy , Aydin Carolyn , Brown Diane S. 
Published: May 2008

Objective. To compare alternative measures of nurse staffing and assess the relative strengths and limitations of each measure.Data Sources/Study Setting. Primary and secondary data from 2000 and 2002 on hospital nurse staffing from the American Hospital Association, California Office of Statewide Health Planning and Development, California Nursing Outcomes Coalition, and the California Workforce Initiative Survey.Study Design. Hospital‐level and unit‐level data were compared using summary statistics, t‐tests, and correlations.Data Collection/Extraction Methods. Data sources were matched for each hospital. When possible, hospital units or types of units were matched within each hospital. Productive nursing hours and direct patient care hours were converted to full‐time equivalent employment and to nurse‐to‐patient ratios to compare nurse staffing as measured by different surveys.Principal Findings. The greatest differences in staffing measurement arise when unit‐level data are compared with hospital‐level aggregated data reported in large administrative databases. There is greater dispersion in the data obtained from publicly available, administrative data sources than in unit‐level data; however, the unit‐level data sources are limited to a select set of hospitals and are not available to many researchers.Conclusions. Unit‐level data collection may be more precise. Differences between databases may account for differences in research findings.
A probable case of hepatitis B virus transfusion transmission revealed after a 13‐month‐long window period
Authors: Wendel Silvano , Levi José E. , Biagini Silvana , Candotti Daniel , Allain Jean‐Pierre 
Published: May 2008

BACKGROUND: Transfusion‐transmitted hepatitis B virus (HBV) infection in recipients with drug‐related immunodeficiency is rarely described in endemic areas. Hepatitis B surface antigen (HBsAg)–negative infectious donor blood can be identified by sensitive nucleic acid testing (NAT). Two immunodeficient patients who received blood components from a single seronegative blood donor subsequently found to contain HBV DNA are described.MATERIALS AND METHODS: Multiple samples from the implicated donor and the two recipients were tested for HBV serologic and molecular markers. HBV genome fragments were amplified, sequenced, and phylogenetically analyzed.RESULTS: The implicated donation had low‐level HBV DNA due to the donor being in the window period before the donor's seroconversion. Recipient 1 had been vaccinated to HBV and carried anti‐HBs but remained negative for all other HBV markers until she developed acute hepatitis B (viral load 2.7 × 108 IU/mL and alanine aminotransferase [ALT] level 1744 IU/L) 13 months after transfusion of red cells. Identical HBV sequences from both donor and recipient provided evidence of transfusion‐related infection. Recipient 2, who received platelets from the same donation while receiving major chemotherapy, remained uninfected.CONCLUSIONS: In unusual circumstances, HBV incubation time can be considerably prolonged. Both active and passive neutralizing antibodies to HBV likely delayed, but did not prevent, acute infection when the immune system was impaired. HBV NAT may have interdicted the infectious unit, although the donation viral load could not be quantified and odds of detection calculated.