bluuebunny/crossref_metadata_2025_split huggingface dataset:
Parents evaluation of the processes of care in child rehabilitation: a reliability study of the Norwegian translation of MPOC‐20
Authors: Hagen A.‐K. , Bjorbækmo W. S. 
Published: December 2010

AbstractBackground  The objective was to assess the reliability of the Norwegian translation of the Canadian Measure of Processes of Care‐20 (MPOC‐20) questionnaire for use in child rehabilitation in Norway. MPOC‐20 is a standardized questionnaire with 20 items and 5 scales that assesses to what extent parents experience that the services offered to them and their children are in line with the ideologies and principles of the family‐centred service (FCS).Methods  The translation of MPOC‐20 was performed according to international standards for translation of questionnaires. A pilot study was carried out with representatives from four patient advocacy organizations. A test–retest reliability study was performed. MPOC‐20 (N) was sent to the participants twice with 2‐ to 6‐week intervals. Participants were 36 parents of 25 children aged 0–18 years. Response rate was 41.8%.Results  The intraclass correlations (ICCs) for test–retest scores ranged 0.78–0.86. The internal consistency expressed as Cronbach's alpha ranged 0.62–0.93; the score was high for four of the scales and acceptable for the fifth ‘Providing specific information’. These findings strongly suggest that the five scales of MPOC‐20 (N) have good test–retest reliability. Average scores of each scale did not differ significantly between the first and second rating, indicating that the average scores of the ratings are stable.Conclusions  The Measure of Processes of Care‐20 (N) appears to be reliable and useful in the Norwegian context, and can be recommended for evaluation of the processes of child rehabilitation1 in Norway.
New homozygous SPINK5 mutation, p.Gln333X, in a Turkish pedigree with Netherton syndrome
Authors: Fong K. , Akdeniz S. , Isi H. , Taskesen M. , McGrath J. A. , Lai-Cheong J. E. 
Published: December 2010

Summary
Netherton syndrome (NS) is a rare autosomal recessive genodermatosis caused by loss-of-function mutations in the SPINK5 gene. The clinical features include congenital ichthyosis, trichorrhexis invaginata and atopy. In this study, we report a new homozygous SPINK5 mutation, p.Gln333X, responsible for NS in affected members of two closely related Turkish families, and provide an overview of the genotype–phenotype correlation in this condition.
Functional characterization of Rho GTPases in Aspergillus niger uncovers conserved and diverged roles of Rho proteins within filamentous fungi
Authors: Kwon Min Jin , Arentshorst Mark , Roos Eelke D. , van den Hondel Cees A. M. J. J. , Meyer Vera , Ram Arthur F. J. 
Published: December 2010

SummaryRho GTPases are signalling molecules regulating morphology and multiple cellular functions including metabolism and vesicular trafficking. To understand the connection between polarized growth and secretion in the industrial model organism Aspergillus niger, we investigated the function of all Rho family members in this organism. We identified six Rho GTPases in its genome and used loss‐of‐function studies to dissect their functions. While RhoA is crucial for polarity establishment and viability, RhoB and RhoD ensure cell wall integrity and septum formation respectively. RhoC seems to be dispensable for A. niger. RacA governs polarity maintenance via controlling actin but not microtubule dynamics, which is consistent with its localization at the hyphal apex. Both deletion and dominant activation of RacA (RacG18V) provoke an actin localization defect and thereby loss of polarized tip extension. Simultaneous deletion of RacA and CftA (Cdc42) is lethal; however, conditional overexpression of RacA in this strain can substitute for CftA, indicating that both proteins concertedly control actin dynamics. We finally identified NoxR as a RacA‐specific effector, which however, is not important for apical dominance as reported for A. nidulans but for asexual development. Overall, the data show that individual Rho GTPases contribute differently to growth and morphogenesis within filamentous fungi.
High‐resolution esophageal pressure topography is superior to conventional sleeve manometry for the detection of transient lower esophageal sphincter relaxations associated with a reflux event
Authors: Rohof W. O. A. , Boeckxstaens G. E. E. , Hirsch D. P. 
Published: December 2010

AbstractBackground  Transient lower esophageal sphincter relaxations (TLESRs) are the main mechanism underlying gastro‐esophageal reflux and are detected during manometric studies using well defined criteria. Recently, high‐resolution esophageal pressure topography (HREPT) has been introduced and is now considered as the new standard to study esophageal and lower esophageal sphincter (LES) function. In this study we performed a head‐to‐head comparison between HREPT and conventional sleeve manometry for the detection of TLESRs.Methods  A setup with two synchronized MMS‐solar systems was used. A solid state HREPT catheter, a water‐perfused sleeve catheter, and a multi intraluminal impedance pH (MII‐pH) catheter were introduced in 10 healthy volunteers (M6F4, age 19–56). Subjects were studied 0.5 h before and 3 h after ingestion of a standardized meal. Tracings were blinded and analyzed by the three authors according to the TLESR criteria.Key Results  In the HREPT mode 156 TLESRs were scored, vs 143 during sleeve manometry (P = 0.10). Hundred and twenty‐three TLESRs were scored by both techniques. Of all TLESRs (177), 138 were associated with reflux (78%). High‐resolution esophageal pressure topography detected significantly more TLESRs associated with a reflux event (132 vs 119, P = 0.015) resulting in a sensitivity for detection of TLESRs with reflux of 96% compared to 86% respectively. Analysis of the discordant TLESRs associated with reflux showed that TLESRs were missed by sleeve manometry due to low basal LES pressure (N = 5), unstable pharyngeal signal (N = 4), and residual sleeve pressure >2 mmHg (N = 10).Conclusions & Inferences  The HREPT is superior to sleeve manometry for the detection of TLESRs associated with reflux. However, rigid HREPT criteria are awaited.
Rice interspecies hybrids show precocious or delayed developmental transitions in the endosperm without change to the rate of syncytial nuclear division
Authors: Ishikawa Ryo , Ohnishi Takayuki , Kinoshita Yuki , Eiguchi Mitsugu , Kurata Nori , Kinoshita Tetsu 
Published: December 2010

SummaryIn angiosperms, interspecific crosses often display hybrid incompatibilities that are manifested as under‐proliferation or over‐proliferation of endosperm. Recent analyses using crosses between Arabidopsis thaliana and its related species with different ploidy levels have shown that interspecific hybridization causes delayed developmental transition and increased mitotic activity in the endosperm. In this study, we investigated endosperm development in interspecific crosses between diploid Oryza species. In a cross between female O. sativa and male O. punctata, we found that the hybrid endosperm was reduced in size and this cross was associated with precocious developmental transition. By contrast, the cross between O. sativa and O. longistaminata generated enlarged hybrid endosperm at the mid‐point of seed development and this cross was associated with delayed developmental transition. Subsequently, the hybrid endosperm displayed a shriveled appearance at the seed maturation stage. We found that the accumulation of storage products and the expression patterns of several marker genes were also altered in the hybrid endosperm. By contrast, the rate of syncytial mitotic nuclear divisions was not significantly affected. The gene OsMADS87 showed a maternal origin‐specific expression pattern in rice endosperm, in contrast to its Arabidopsis homologue PHERES1, which shows paternal origin‐specific expression. OsMADS87 expression was decreased or increased depending on the type of developmental transition change in the hybrid rice endosperm. Our results indicate that one of the interspecies hybridization barriers in Oryza endosperm is mediated by precocious or delayed developmental alterations and de‐regulation of OsMADS87, without change to the rate of syncytial mitotic nuclear division in the hybrid endosperm.
Small RNAs from MITE‐derived stem‐loop precursors regulate abscisic acid signaling and abiotic stress responses in rice
Authors: Yan Yongsheng , Zhang Yuman , Yang Kun , Sun Zongxiu , Fu Yaping , Chen Xiaoying , Fang Rongxiang 
Published: December 2010

SummarySmall silencing RNAs (sRNAs) are non‐coding RNA regulators that negatively regulate gene expression by guiding mRNA degradation, translation repression or chromatin modification. Plant sRNAs play crucial roles in various developmental processes, hormone signaling, immune responses and adaptation to a variety of abiotic stresses. miR441 and miR446 were previously annotated as microRNAs (miRNAs) because their precursors can form typical stem‐loop structures, but are not considered as real miRNAs because of inconsistency with some ancillary criteria of the recent guidelines for annotation of plant miRNAs. We tentatively rename them small interfering (si)R441 and siR446, respectively, in this study. It has recently been shown that the precursors of siR441 and siR446 might originate from the miniature inverted‐repeat transposable element (MITE) Stowaway1. In this report, we show that, in contrast with Dicer‐like (DCL)3‐ and RNA‐dependent RNA polymerase (RDR)2‐dependent MITE‐derived ra‐siRNAs, siR441 and siR446 are processed by OsDCL3a but independent of OsRDR2, indicating that siR441 and siR446 are generated from single‐stranded stem‐loop precursors. We also show that abscisic acid (ABA) and abiotic stresses downregulate the expression of siR441 and siR446 but, surprisingly, increase the accumulation of their precursors in rice plants, implying that processing of siRNA precursors is inhibited. We provide evidence to show that this defective processing is due to increased precursor accumulation per se, possibly by intermolecular self‐pairing of the processing intermediate sequences, thus hindering their normal processing. Functional examinations indicate that siR441 and siR446 are positive regulators of rice ABA signaling and tolerance to abiotic stress, possibly by regulating MAIF1 expression.
TFL2/LHP1 is involved in auxin biosynthesis through positive regulation of YUCCA genes
Authors: Rizzardi Kristina , Landberg Katarina , Nilsson Lars , Ljung Karin , Sundås‐Larsson Annika 
Published: December 2010

Summary TERMINAL FLOWER2 (TFL2) is the plant homologue of metazoan HETEROCHROMATIN PROTEIN1 (HP1) protein family. It is known that, unlike most HP1 proteins, TFL2 does not primarily localize to heterochromatin; instead it functions in regulation of specific genes in euchromatic regions. We show that the tfl2 mutant has a lower rate of auxin biosynthesis, resulting in low levels of auxin. In line with this, tfl2 mutants have lower levels of expression of auxin response genes and retain an auxin response. The reduced rate of auxin biosynthesis in tfl2 is correlated to the down‐regulation of specific genes in the tryptophan‐dependent auxin biosynthesis pathway, a sub‐set of the YUCCA genes. In vivo, TFL2 is targeted to a number of the YUCCA genes in an auxin‐dependent fashion revealing a role of TFL2 in auxin regulation, probably as a component of protein complexes affecting transcriptional control.
Transcription‐dependence of histone H3 lysine 27 trimethylation at the Arabidopsis polycomb target gene FLC
Authors: Buzas Diana Mihaela , Robertson Masumi , Finnegan E. Jean , Helliwell Chris A. 
Published: December 2010

SummaryThe FLC gene encodes a MADS box repressor of flowering that is the main cause of the late‐flowering phenotype of many Arabidopsis ecotypes. Expression of FLC is repressed by vernalization; maintenance of this repression is associated with the deposition of histone 3 K27 trimethylation (H3K27me3) at the FLC locus. However, whether this increased H3K27me3 is a consequence of reduced FLC transcription or the cause of transcriptional repression is not well defined. In this study we investigate the effect of changes in transcription rate on the abundance of H3K27me3 in the FLC gene body, a chromatin region that includes sequences required to maintain FLC repression following vernalization. We show that H3K27me3 is inversely correlated with transcription across the FLC gene body in a range of ecotypes and mutants with different flowering times. We demonstrate that the FLC gene body becomes marked with H3K27me3 in the absence of transcription. When transcription of the gene body is directed by an inducible promoter, H3K27me3 is removed following activation of transcription and H3K27me3 is added after transcription is decreased. The rate of addition of H3K27me3 to the FLC transgene following inactivation of transcription is similar to that observed in the FLC gene body following vernalization. Our data suggest that reduction of FLC transcription during vernalization leads to an increase of H3K27me3 levels in the FLC gene body that in turn maintains FLC repression.
The intracellular Arabidopsis COPT5 transport protein is required for photosynthetic electron transport under severe copper deficiency
Authors: Garcia‐Molina Antoni , Andrés‐Colás Nuria , Perea‐García Ana , del Valle‐Tascón Secundino , Peñarrubia Lola , Puig Sergi 
Published: December 2010

SummaryCopper is an essential micronutrient that functions as a redox cofactor in multiple plant processes, including photosynthesis. Arabidopsis thaliana possesses a conserved family of CTR‐like high‐affinity copper transport proteins denoted as COPT1‐5. COPT1, the only family member that is functionally characterized, participates in plant copper acquisition. However, little is known about the function of the other Arabidopsis COPT proteins in the transport and distribution of copper. Here, we show that a functional fusion of COPT5 to the green fluorescent protein localizes in Arabidopsis cells to the prevacuolar compartment. Plants defective in COPT5 do not exhibit any significant phenotype under copper‐sufficient conditions, but their growth is compromised under copper limitation. Under extreme copper deficiency, two independent copt5 knockout mutant lines exhibit severe defects in vegetative growth and root elongation, low chlorophyll content, and impairment in the photosynthetic electron transfer. All these phenotypes are rescued when the wild‐type copy of the COPT5 gene is retransformed into a copt5 knockout line or when copper, but not other metals, are added to the medium. COPT5 is expressed in vascular tissues, with elevated levels in roots. Taken together, these results suggest that COPT5 plays an important role in the plant response to environmental copper scarcity, probably by remobilizing copper from prevacuolar vesicles, which could act as internal stores or recycling vesicles to provide the metal cofactor to key copper‐dependent processes such as photosynthesis.
The Clavata2 genes of pea and Lotus japonicus affect autoregulation of nodulation
Authors: Krusell Lene , Sato Naoto , Fukuhara Izumi , Koch Bjørn E. V. , Grossmann Christina , Okamoto Satoru , Oka‐Kira Erika , Otsubo Yoko , Aubert Grégoire , Nakagawa Tomomi , Sato Shusei , Tabata Satoshi , Duc Gerard , Parniske Martin , Wang Trevor L. , Kawaguchi Masayoshi , Stougaard Jens 
Published: December 2010

SummaryThe number of root nodules developing on legume roots after rhizobial infection is controlled by the plant shoot through autoregulation and mutational inactivation of this mechanism leads to hypernodulation. We have characterised the Pisum sativum (pea) Sym28 locus involved in autoregulation and shown that it encodes a protein similar to the Arabidopsis CLAVATA2 (CLV2) protein. Inactivation of the PsClv2 gene in four independent sym28 mutant alleles, carrying premature stop codons, results in hypernodulation of the root and changes to the shoot architecture. In the reproductive phase sym28 shoots develops additional flowers, the stem fasciates, and the normal phyllotaxis is perturbed. Mutational substitution of an amino acid in one leucine rich repeat of the corresponding Lotus japonicus LjCLV2 protein results in increased nodulation. Similarly, down‐regulation of the Lotus Clv2 gene by RNAi mediated reduction of the transcript level also resulted in increased nodulation. Gene expression analysis of LjClv2 and Lotus hypernodulation aberrant root formation Har1 (previously shown to regulate nodule numbers) indicated they have overlapping organ expression patterns. However, we were unable to demonstrate a direct protein–protein interaction between LjCLV2 and LjHAR1 proteins in contrast to the situation between equivalent proteins in Arabidopsis. LjHAR1 was localised to the plasma membrane using a YFP fusion whereas LjCLV2‐YFP localised to the endoplasmic reticulum when transiently expressed in Nicotiana benthamiana leaves. This finding is the most likely explanation for the lack of interaction between these two proteins.
The significance of anticardiolipin antibody and immunologic abnormality in livedoid vasculitis
Authors: Feng Su‐ying , Jin Pei‐ying , Shao Chang‐geng 
Published: December 2010

AbstractObjective  To evaluate the significance of anticardiolipin and immunologic abnormality in the livedoid vasculitis (LV).Methods  30 patients with biopsy‐proven LV and 30 normal controls involved in the study. Indirect immunofluorescence, immunoblot, and ELISA were used for detecting antinuclear antibody (ANA), circulating immune complex, immune globulin, anticardiophospholipin antibody (ACA), and anti‐β2GP1.Results  ANA was positive in four patients with LV, and among them, two patients were diagnosed as Systemic Lupus Erythematosus (SLE) later. Addition to the two SLE patients, the level of ENA and immunoglobulin were normal in the rest of patients. Anticardiolipin antibodies were present in 13 (43.33%), and β2GP1 was present in nine (30%) of 30 patients. There were significant differences between LV and controls.Conclusions  ACA is one of important pathogenesis of LV. Numerous heterogeneous coagulation abnormalities and thrombogenesis may involve the LV.
Causes of death among active leprosy patients in China
Authors: Shen Jianping , Liu Musang , Zhou Min , Li Wenzhong 
Published: December 2010

AbstractWe analyzed the causes of death among active leprosy patients in China, to better understand the disease and improve the quality of services, by performing a retrospective study involving 24 provinces of China. Information about patients with active leprosy who were not clinically cured and died between January 1, 2000, and December 31, 2005, was collected by professionals at county levels. A total of 524 deaths were analyzed. The leading cause of death was suicide. There were 86 patients (16%) who died at a mean of 21 ± 19 months after starting multi‐drug therapy (MDT). The second and third leading causes of death were cardiovascular disease and organ failure associated with advanced age, respectively. Two hundred and twenty‐one patients (42%) died within one year of beginning MDT. The second month of MDT was the riskiest for newly treated patients; approximately 20% of patients succumbed to liver failure, 33% to dapsone (diaminodiphenylsulfone/DDS) allergy, and 27% to renal insufficiency during this period. Among 143 deaths related to leprosy, 37 (26%) occurred within three months of starting MDT. We recommend that newly diagnosed patients should be provided with no more than two months of MDT blister packs.
Assessment of osteoporosis in psoriasis with and without arthritis: correlation with disease severity
Authors: Attia Enas A. S. , Khafagy Ayman , Abdel‐Raheem Sameh , Fathi Sahar , Saad Abeer A. 
Published: December 2010

AbstractBackground  The most frequent extracutaneous association with psoriasis is arthritis. Because proinflammatory cytokines are increased in psoriasis, patients with this disease may be more prone to osteoporosis than the healthy individuals.Methods  We evaluated 50 patients with psoriasis, with or without psoriatic arthritis (PsA), for the presence and degree of osteoporosis by performing dual energy x‐ray absorptiometry (DEXA) and obtaining serum osteoprotegrin (OPG) levels. In addition, we correlated these results with the extent of skin and joint disease. Psoriasis area and severity index (PASI) was determined in all 50 patients with psoriasis, and total joint score (TJS) was recorded in the 16 patients who also had PsA. Results of DEXA and serum OPG were also obtained for 20 healthy individuals who served as controls.Results  Osteoprotegrin level was significantly increased in psoriasis patients (with or without PsA) vs. controls. However, DEXA revealed that PsA patients had a higher degree of osteoporosis in the femur neck and wrist. In PsA patients, TJS correlated positively with both disease duration and PASI but correlated negatively with Z score of the femur.Conclusion  Psoriasis patients with or without arthritis may suffer from osteoporosis as evidenced by significantly increased serum OPG. Prolonged and extensive cutaneous disease is an important risk factor for the development and severity of PsA. Patients with a greater number of affected joints are at higher risk of osteoporosis.
Measuring performance in skin cancer practice: the SCARD initiative
Authors: Rosendahl Cliff , Hansen Craig , Cameron Alan , Bourne Peter , Wilson Tobias , Cook Ben , Baker Martin , Keir Jeff , Dicker Tony , Reid Mike , Williamson Richard , Weedon David , Soyer H. Peter , Youl Philippa H. , Wilkinson David 
Published: December 2010

AbstractBackground  The Skin Cancer College of Australia and New Zealand (SCCANZ) has developed a unique project named SCARD – the Skin Cancer Audit and Research Database. Designed initially as a self‐audit tool for primary care skin cancer practitioners, SCARD acts as a tracking tool to enhance practice safety, and it also creates practice performance reports. Pooling of de‐identified data enables participating practitioners to confidentially compare their own practice to that of their peers. Additionally, this creates a large database with significant research potential, as SCARD records for every lesion de‐identified practitioner and patient data, and extensive details of location, provisional and histological diagnosis, and the procedure(s) performed in its treatment.Methods  Preliminary data collected in the database have been presented in this study.Results  An initial pool of data from 177 practitioners contains 77,553 specimens from 41,006 individual patients.Conclusions  The data presented are being analyzed for further studies, and additional data continues to be collected from this ongoing project. SCARD is a useful tool at practice level, and substantial uptake by Australian primary care skin cancer practitioners has provided a unique opportunity for research into skin cancer and its management. SCCANZ, a professional college of predominantly primary care medical practitioners, with a commitment to the management of skin cancer in Australia and New Zealand, has formed a partnership with the School of Medicine at the University of Queensland to ensure that these data are managed and analyzed appropriately.