bluuebunny/crossref_metadata_2025 huggingface dataset:
Mucopolysaccharidosis type VI: Identification of novel mutations on the arylsulphatase B gene in South American patients
Authors: Petry M. F. G. , Nonemacher K. , Sebben J. C. , Schwartz I. V. D. , Azevedo A. C. M. , Burin M. G. , De Rezende A. R. , Kim C. A. , Giugliani R. , Leistner‐Segal S.
Published: January 2006

Mucopolysaccharidosis type VI (Maroteaux–Lamy syndrome, MPS VI) is an autosomal recessive disorder caused by deficiency of N‐acetylgalactosamine‐4‐sulphatase (ARSB),which leads to the lysosomal accumulation and excretion of dermatan sulphate (DS). In this study, 13 unrelated MPS VI patients (12 Brazilian and 1 Chilean) were investigated regarding the identification of the ARSB gene mutations using PCR, SSCP and sequencing. The exons with altered mobility on SSCP were sequenced, as well as all the exons of patients with no SSCP alteration. Seven novel mutations were identified: D59N, L72R, Q88H, P93S, R197X, 1279delA and c.1143−8T > G. The previously reported mutations 1533del23, R315Q and 427delG were found in six, three and two alleles respectively. The other mutations already reported, S384N and G144R, were found in only one allele. In addition, three polymorphisms previously described (V358M, V376M and P397P) were detected in the patients analysed. Our findings are in agreement with the literature confirming the great genetic heterogeneity associated with MPS VI.
A novel deletion in a Pearson syndrome infant with hypospadias and cleft lip and palate
Authors: Lohi O. , Kuusela A. L. , Arola M.
Published: January 2006

Pearson syndrome is a rare multiorgan mitochondrial disorder that causes substantial disability and usually leads to premature death. We describe an infant with Pearson syndrome who showed, in addition to the typical features of the syndrome, cleft lip and palate and hypospadias.
The intake of total protein, natural protein and protein substitute and growth of height and head circumference in Dutch infants with phenylketonuria
Authors: Hoeksma M. , Van Rijn M. , Verkerk P. H. , Bosch A. M. , Mulder M. F. , de Klerk J. B. C. , De Koning T. J. , Rubio‐Gozalbo E. , De Vries M. , Sauer P. J. J. , van Spronsen F. J.
Published: January 2006

In a previous study, Dutch children with phenylketonuria (PKU) were found to be slightly shorter than their healthy counterparts. In the literature, it has been hypothesized that a higher protein intake is necessary to optimize growth in PKU patients. The study aimed to investigate whether protein intake (total, natural and protein substitute) in this group might be an explanatory factor for the observed growth. Growth of height and head circumference and dietary data on protein intake (total, natural and protein substitute) from 174 Dutch PKU patients born between 1974 and 1996 were analysed retrospectively for the patients' first 3 years of life. Analyses were corrected for energy intake during the first year of life and for the clinical severity of the deficiency of phenylalanine hydroxylase by means of plasma phenylalanine concentration at birth. Neither protein nor energy intake correlated with height growth. A positive, statistically significant relation between head circumference growth and natural protein and total protein intake was found, but not with the intake of the protein substitute or energy. Therefore, this study suggests that improvement of the protein substitute rather than an increase of total protein intake may be important in optimizing head circumference growth in PKU patients.
The knowledge gap in expanded newborn screening: Survey results from paediatricians in Massachusetts
Authors: Gennaccaro M. , Waisbren S. E. , Marsden D.
Published: January 2006

Massachusetts currently offers an optional expanded newborn screening programme that tests for 20 biochemical genetic disorders in addition to the mandated newborn screening tests, including phenylketonuria (PKU) and nine other biochemical genetic disorders. We conducted a mail survey of 550 paediatricians listed in the 2000Massachusetts Healthcare Directory to determine paediatricians' preparedness in discussing expanded newborn screening and its results with families, and to determine in what specific format physicians in Massachusetts would prefer to receive educational materials and updates. Of surveys mailed, 35% (190/550) were returned within the allotted 3 weeks: 25 paediatricians (14%) were unaware of expanded newborn screening; 78 respondents (42%) indicated feeling less than prepared talking about test results with families; 100 paediatricians (54%) indicated a lack of information about metabolic disorders; 134 (73%) preferred information sent in postal mailings, 62 (34%) preferred grand rounds, 60 (33%) preferred educational seminars, and 58 (32%) preferred websites. Other formats receiving preferences of less than 30% included e‐mail (27%), phone calls (8%), video (6%), and distance learning (1%). Paediatricians are ill‐prepared for expanded newborn screening for biochemical genetic disorders. To address this problem, paediatricians in Massachusetts indicated a preference for unsolicited periodic mailings including short reviews and brochures.
Body composition in children with galactosaemia
Authors: Panis B. , Forget P. Ph. , Nieman F. H. , van Kroonenburgh M. J. P. G. , RubioGozalbo M. E.
Published: January 2006

Body composition in classical galactosaemia has not been studied. Patients with classical galactosaemia, an inherited disorder of galactose metabolism caused by deficiency of galactose‐1‐phosphate uridyltransferase (GALT, EC 2.7.7.10), might be at risk for an abnormal body composition because of intrinsic factors related to galactosaemia and/or diet‐related factors. The aim of this study was to evaluate the body composition of children with classical galactosaemia. The studied population was a previously reported group of classical galactosaemia patients (13 male and 27 female, ages 3–17 years) with decreased height, weight, weight‐for‐height and insulin‐like growth factor‐I (IGF‐I) Z‐scores. Body composition data were obtained by dual‐energy X‐ray absorptiometry (DXA). In order to correct for height, fat mass (FM) and lean tissue mass (LTM) were divided by squared height. Mid‐parental target height Z‐scores were assessed and compared to actual height Z‐scores. Linear and multiple regression analysis were done to investigate the relationship between body composition and IGF‐I, dietary intake and growth data. We found decreased height Z‐scores when compared to mid‐parental target height Z‐scores. Mean scores for FM and LTM (both adjusted for height) were decreased. LTM (adjusted for height) and height Z‐score were correlated with IGF‐I Z‐score. FM (adjusted for height) was correlated with soy intake. No correlation was found between soy intake and IGF‐I Z‐score. In this limited group of patients, height is decreased and body composition is abnormal. The decreased levels of IGF‐I and/or soy nutrition might play a role in these findings.
External quality assurance programme for enzymatic analysis of lysosomal storage diseases: A pilot study
Authors: Ruijter G. J. G. , Boer M. , Weykamp C. W. , De Vries R. , van den Berg I. , Janssens‐Puister J. , Niezen‐Koning K. , Wevers R. A. , Poorthuis B. J. H. M. , van Diggelen O. P.
Published: January 2006

Inborn errors of metabolism are rare and laboratories performing diagnostic tests in this field must participate in external quality assurance (EQA) schemes to demonstrate their competence and also to maintain sufficient experience with patient material. EQA schemes for metabolite analyses are available (ERNDIM), but corresponding EQA schemes for enzyme analyses are nonexistent. In this paper we describe a pilot study on lysosomal enzyme testing by four centres in The Netherlands. Quantitative aspects of EQA were studied by interlaboratory comparison of activities of six lysosomal enzymes in a series of buffy coat samples. Interlaboratory variance was enormous. To reduce variance caused by methodological differences, participants reported enzyme activities relative to mean normal values. β‐D‐Galactosidase activities compared well between the participating laboratories (average interlaboratory CV 13%), but for other enzymes large differences were observed, e.g. sphingomyelinase (average CV 38%). Diagnostic proficiency was tested with cultured fibroblasts. In 45 out of a total of 48 tests (12 cell lines, 4 participants) the correct diagnosis was accomplished on the basis of merely biochemical investigations, i.e. without clinical data of the patients. In a survey using blood of a late‐onset Pompe disease patient, less conclusive results were obtained. A stable enzyme source was developed for easy distribution. Most lysosomal enzymes were stable upon lyophilization of leukocyte homogenates and during subsequent storage of the freeze‐dried material at room temperature, in particular when cryolyoprotectant was added. Shipment of such lyophilized samples is simple and cheap and ideal for an EQA scheme. Our study shows that an EQA programme for enzymatic testing of lysosomal storage diseases is necessary to accomplish reliable diagnostic procedures for lysosomal storage diseases. We recommend that EQA for lysosomal enzymes be implemented through ERNDIM.
Anxiety and depression in the acute porphyrias
Authors: Millward L. M. , Kelly P. , King A. , Peters T. J.
Published: January 2006

A previous study of self‐rated psychosocial aspects in patients with acute porphyria found that depression, and particularly anxiety, is more common in porphyria patients than in the general population or general medical outpatient attenders. Nearly half of the sample (46%) reported at least some problem with anxiety and/or depression: anxiety caseness was 26% and depression caseness was 13%. This paper extends our previous observations and investigates further the associations between porphyria and anxiety, depression and general mental health in 90 patients (58 acute intermittent porphyria, 32 variegate porphyria). The findings of this study confirm that anxiety is raised in patients with acute intermittent porphyria and with variegate porphyria, in both males and females, compared to the normative population and, using a series of questionnaires exploring the physical and psychosocial features of anxiety, that this anxiety is experienced as a ‘relatively stable personality trait’, rather than a ‘transitory emotional state’ (i.e. intrinsic rather than secondary to the porphyria).
Clinical, biochemical, magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (1H MRS) findings in a fourth case of combined D‐ and L‐2 hydroxyglutaric aciduria
Authors: Read M.‐H. , Bonamy C. , Laloum D. , Belloy F. , Constans J. M. , Guillois B. , Kottler M.‐L. , Verhoeven N. M. , Jakobs C.
Published: January 2006

We report the fourth case of combined d‐and L‐2‐hydroxyglutaric aciduria presenting with neonatal encephalopathy and subependymal cysts.
Older People's Experiences of Loneliness in the UK: Does Gender Matter?
Authors: Victor Christina R. , Scambler Sasha J. , Marston Louise , Bond John , Bowling Ann
Published: January 2006

The extent and nature of loneliness in later life does not show a consistent relationship with gender. Our study investigates whether there are differences in the nature and extent of loneliness amongst older men and women in contemporary Britain.

Loneliness was measured using a self-report four-point scale in a nationally representative survey of people aged 65+ living in the community.

Survey response rate was 77 per cent and the sample of 999 approximates to that of the general population. Approximately half of our sample 53 per cent were women. Compared with males in the sample women were significantly more likely to be widowed, live alone and have direct contact with friends and relatives. Preliminary analysis identified statistically significant differences between men and women in and self-reported loneliness (and changes over the previous decade). Ordered logistic regression, indicated that gender was no longer independently associated with loneliness once the confounding influences of marital status, age and living arrangement were excluded.

The overall self-reported prevalence of severe loneliness shows little difference between men and women, challenging the stereotype that loneliness is a specifically female experience.
Kommunikative Regulation und sprachlicher Ausdruck von Ist-Soll-Diskrepanzen in mündlichen und schriftlichen Äußerungen
Authors: Buhl Heike M. , Hofer Manfred , Lege Thomas
Published: January 2006

Zusammenfassung. Der Regulationstheorie der Sprachproduktion folgend gibt eine Ist-Soll-Diskrepanz jenseits tolerierbarer Grenzen ggf. den Anstoß zum Sprachproduktionsprozess, der in eine Äußerung mündet (vgl. Herrmann & Grabowski, 2003 ). Wir nehmen an, dass die Stärke der Ist-Soll-Diskrepanz die Häufigkeit der Verbalisierung von Soll- und Ist-Informationen in der sprachlichen Äußerung beeinflusst. Bei starkem Ziel werden mehr Soll-Informationen erwartet als bei schwachem Ziel. In zwei Rollenspielexperimenten wurden Äußerungen Jugendlicher in Konfliktgesprächen mit der Mutter erhoben, wobei die Stärke der Ist-Soll-Diskrepanz, operationalisiert über die Bedeutsamkeit des Zieles in einer Konfliktsituation, variiert wurde. Um Einflüssen der Kommunikationssituation nachzugehen, wurde das erste Experiment mündlich (N = 53), das zweite schriftlich (N = 131) durchgeführt. Insgesamt wurden schriftlich mehr Soll-Informationen verbalisiert. Im mündlichen Rollenspielexperiment fanden sich wie erwartet bei starkem Ziel mehr Soll-Informationen als bei schwachem Ziel. Im schriftlichen Rollenvorstellungsexperiment dagegen waren bei starkem Ziel sogar weniger Soll-Informationen zu verzeichnen, was unter Berücksichtigung situationaler Unterschiede zwischen mündlicher und schriftlicher Sprachproduktion diskutiert wird.
Wie effektiv sind systematische Rückmeldungen des Therapieverlaufs an den Therapeuten?
Authors: Berking Matthias , Orth Ulrich , Lutz Wolfgang
Published: January 2006

Zusammenfassung. Theoretischer Hintergrund: Die systematische Rückmeldung des Therapieverlaufs an den Therapeuten erwies sich in mehreren Studien an ambulant behandelten, überwiegend gering beeinträchtigten Patienten als effektives Mittel, um die Wirksamkeit psychotherapeutischer Behandlungen für potenziell problematische Patienten zu steigern. Fragestellung: Lassen sich diese Befunde auch in einem stationär-verhaltenstherapeutischen Setting replizieren und inwieweit sind sie wirklich auf misserfolgsgefährdete Patienten beschränkt? Methode: In einem randomisierten Gruppenvergleich (N = 118) erhielt die Hälfte der Therapeuten ein systematisches Feedback über den Therapieverlauf. Ergebnisse: Die Patienten in der Feedback-Bedingung verbesserten sich bei allen untersuchten Therapieerfolgsmaßen stärker als die Patienten der Kontrollgruppe. Schlussfolgerung: Ein systematisches Feedback des Therapieverlaufs an den Therapeuten führt zu einer Verbesserung der Effektivität stationär-verhaltenstherapeutischer Behandlungen. Zukünftige Forschung sollte nach Wegen suchen, die monetären und psychologischen Kosten von Feedbacksystemen zu reduzieren, um deren Einsatz in Settings der Routineversorgung zu erleichtern.
Klinische Cyberpsychologie: Die Anwendung virtueller Realitäten in der psychologischen Diagnostik und Behandlung
Authors: Huber Helmuth P.
Published: January 2006

Zusammenfassung. Theoretischer Hintergrund und Fragestellung: Eine von Jahr zu Jahr steigende Anzahl von Publikationen signalisiert die Entstehung eines neuen Gebiets der Klinischen Psychologie, das man “Klinische Cyberpsychologie“ nennen könnte. Die vorliegende Arbeit versucht vor allem einen Überblick über die neuesten Ergebnisse der Anwendung “virtueller Realitäten“ (VR) in der psychologischen Diagnostik und Behandlung zu geben. Methode: Es wurden drei Ansätze unterschieden: a) Die E-Therapie, b) die VR-Therapie und c) die VR-Teletherapie. Diese Dreiteilung gilt im Wesentlichen auch für den Bereich der Diagnostik, Beratung und Rehabilitation. Ergebnisse und Schlussfolgerungen: Mit Hilfe computergenerierter virtueller Szenarien kann eine wirklichkeitsnahe Reizmodellierung vorgenommen werden, die nicht nur eine gut kontrollierbare Reizdarbietung ermöglicht, sondern darüber hinaus auch eine interaktive Gestaltung zulässt. Dies gibt Anlass zur Hoffnung, dass durch den gezielten Einsatz virtueller Systeme die psychologische Diagnostik und Behandlung wesentlich verbessert werden kann.
Analytical expression for the relative dispersion of the cloud droplet size distribution
Authors: Liu Yangang , Daum Peter H. , Yum Seong Soo
Published: January 2006

An analytical expression that relates the relative dispersion (ratio of standard deviation to mean radius) of the cloud droplet size distribution to CCN spectra and updraft velocity is derived from adiabatic growth theory of cloud droplets. Coupled with the Twomey expression for droplet concentration, the analytical expression is used to examine the relationship of relative dispersion to droplet concentration under different combinations of CCN spectra and updraft velocities. These analytical results compare favorably with the corresponding simulations of an adiabatic parcel model. The analytical expression theoretically demonstrates that an increase in aerosol loading (CCN concentration) leads to concurrent increases in the droplet concentration and relative dispersion whereas a larger updraft velocity leads to a higher droplet concentration but a smaller relative dispersion.
Large horizontal gradients in atmospheric CO at the synoptic scale as seen by spaceborne Measurements of Pollution in the Troposphere
Authors: Liu J. , Drummond J. R. , Jones D. B. A. , Cao Z. , Bremer H. , Kar J. , Zou J. , Nichitiu F. , Gille J. C.
Published: January 2006

We have examined the influence of synoptic processes on the distribution of atmospheric CO as observed by the Measurements of Pollution in the Troposphere (MOPITT) satellite instrument. In the MOPITT data, large horizontal gradients in CO, coherent at the synoptic scale, have been observed. The concentration of CO varies rapidly by as much as 50–100% across distances of ∼100 km, forming distinct boundaries in the CO distribution. These can last one to several days and span horizontal distances of 600–1000 km. On average, such events were observed in the MOPITT CO daily images once every 3–4 days over North America in spring and summer 2000. We focused on three case studies over North America in August 2000 to understand the mechanisms responsible for the large gradients in CO. Through an analysis of meteorological data from the National Centers for Environmental Prediction/National Center for Atmospheric Research Reanalysis, parcel trajectory modeling, and global three‐dimensional chemical transport modeling, we found that the large horizontal gradients typically reflect the differential vertical and horizontal transport of air with different chemical signatures. In the first case, the large gradients in CO over North Dakota resulted from the lifting ahead of a cold front that transported boundary layer air enriched with CO from forest fires in Montana, combined with the descent of clean air from the Canadian Prairies behind the front. In the second case, the large gradients over northeastern Texas were produced by the convective lifting over Arkansas of air with high concentrations of CO from the oxidation of volatile organic compounds and the onshore transport of clean air from the Gulf of Mexico. In the third case, we examined an example of outflow of surface pollution from North America by a cyclone. The largest gradients in this case were observed along the boundary between the boundary layer air transported by the warm conveyor belt ahead of the cold front and the clean air transported from the Atlantic by the semipermanent high‐pressure system in the central Atlantic. Our results demonstrate that MOPITT can capture the influence of synoptic processes on the horizontal and vertical distribution of CO. The large gradients in CO observed on synoptic scales represent valuable information that can be exploited to improve our understanding of atmospheric CO. In particular, these results suggest that the MOPITT observations provide a useful data set with which to address a range of issues from air quality on local/regional scales to long‐range transport of pollution on continental/global scales.
Laser generated plasmas characterized under magnetic field
Authors: Ducruet C. , Kornilov N. , de Julián Fernández C. , Givord D.
Published: January 2006

The confinement of laser generated plasmas by a magnetic field is exploited to analyze the properties of plasmas of different materials. The ablation threshold is approximately proportional to Cv(Tmelt−300) (Tmelt=melting temperature, Cv=specific heat). The ablation rate is related to the boiling enthalpy. From aluminum to molybdenum, the increase in the proportion of ions within the plume is discussed by considering the energy per atom available for plasma heating. While negligible in zero magnetic field, ion recombination effects are significant under field. This is attributed to the higher plasma density and to the decrease in the mean energy of the detected ions.
Influence of dielectric properties of a substrate upon plasmon resonance spectrum of supported Ag nanoparticles
Authors: Xu G. , Chen Y. , Tazawa M. , Jin P.
Published: January 2006

Plasmon resonance spectra of supported Ag nanoparticles are studied by depositing the particles on different substrates. It was found that the dielectric properties of the substrates have significant effects on the spectral line shape, except the resonance frequency. Beyond the plasmon resonance band, the spectral shape is mainly governed by the dielectric function, particularly its imaginary part, of the substrate. The plasmon resonance band, on the other hand, may be severely distorted if the substrate is absorbing strongly.
Polarized Raman scattering of multiferroic BiFeO3 epitaxial films with rhombohedral R3c symmetry
Authors: Singh Manoj K. , Jang Hyun M. , Ryu Sangwoo , Jo Moon-Ho
Published: January 2006

Highly (111)-oriented rhombohedral BiFeO3 (BFO) thin films were grown on (111) SrTiO3 substrates by pulsed laser deposition. Polarized Raman-scattering study of the (111)-oriented epitaxial BFO thin film with rhombohedral R3c symmetry was carried out by employing two distinct backscattering geometries. The A1-symmetry transverse-optical [A1(TO)] phonons were selectively isolated from the E-symmetry transverse-optical [E(TO)] phonons by employing Y′(ZZ)Y′ polarization configuration in a novel side-view backscattering. By comparing the Y′(ZZ)Y′ spectrum with the Z(X′X′)Z polarization spectrum in a normal backscattering, we were able to assign most of A1 and E-symmetry normal modes of R3c BFO. In addition, we found that there was a negligible LO-TO splitting in the A1-symmetry normal modes.
Comparative study of the photoresponse from tetracene-based and pentacene-based thin-film transistors
Authors: Choi Jeong-M. , Lee Jiyoul , Hwang D. K. , Kim Jae Hoon , Im Seongil , Kim Eugene
Published: January 2006

We report on the photoresponse from tetracene-based and pentacene-based thin-film transistors (TFTs) with semitransparent NiOx source/drain electrodes and SiO2∕p+-Si substrate. Both organic TFTs have been fabricated with identical channel thickness and device geometry. Compared with pentacene-based TFTs, the tetracene-TFT exhibited superior potentials as a photodetector in the visible and ultraviolet range although it showed a field mobility (μ=0.003cm2∕Vs) which is two orders of magnitude lower than that of the pentacene-based TFT (μ=∼0.3cm2∕Vs). The tetracene-TFT displayed a high photo-to-dark current ratio (Iph∕Idark) of 3×103, while that of the pentacene-TFT was only ∼10.
Photoluminescence from seeded three-dimensional InAs∕GaAs quantum-dot crystals
Authors: Kiravittaya S. , Rastelli A. , Schmidt O. G.
Published: January 2006

We investigate the photoluminescence (PL) properties of three-dimensional InAs∕GaAs quantum-dot (QD) crystals grown on shallow modulated periodic hole arrays patterned on GaAs(001). We find that the PL spectra become narrower and more intense with increasing number of QD layers. A deconvoluted PL linewidth of 14.9 meV is obtained from a defect-free QD crystal consisting of 11 stacked QD layers. The PL spectra obtained for QD crystals containing QD vacancies show significantly broader spectra. The PL peak energy and linewidth of the QDs across the whole pattern (100×100μm2) remain constant within 1.278±0.001eV and 21.0±1.7meV, respectively. From power-dependent PL measurement, we can resolve up to seven excited-state PL peaks confirming the remarkable size homogeneity of our QD crystals. This experimental result can be reasonably fitted by a calculation based on random population theory and on a simple model for the QD confinement potential.
Identification of target actin content and polymerization status as a mechanism of tumor resistance after cytolytic T lymphocyte pressure
Authors: Abouzahr Soraya , Bismuth Georges , Gaudin Catherine , Caroll Oliver , Van Endert Peter , Jalil Abdelali , Dausset Jean , Vergnon Isabelle , Richon Catherine , Kauffmann Audrey , Galon Jérôme , Raposo Graca , Mami-Chouaib Fathia , Chouaib Salem
Published: January 2006

To investigate tumor resistance to T cell lysis, a resistant variant was selected after specific cytolytic T lymphocytes (CTL) selection pressure. Although the resistant variant triggeredperforinandgranzyme Btranscription in specific CTLs, as well as their degranulation, it exhibited a dramatic resistance to cytotoxic T cell killing. It also displayed strong morphological changes with alterations of the actin cytoskeleton. Electron microscopy analysis revealed a loosen interaction between CTLs and the resistant variant despite the formation of apparently normal conjugates. Transcriptional profiling identified a gene expression signature that distinguished sensitive from resistant tumor targets. More notably, we found that actin-related genesephrin-A1andscinderinwere overexpressed in resistant target. Silencing of these genes using RNA interference resulted in a restoration of normal cell morphology and a significant attenuation of variant resistance to CTL killing. Our present study shows that a shift in cytoskeletal organization can be used, by tumor cells, as a strategy to promote their resistance after CTL selection pressure.
Innate antiviral response targets HIV-1 release by the induction of ubiquitin-like protein ISG15
Authors: Okumura Atsushi , Lu Gengshi , Pitha-Rowe Ian , Pitha Paula M.
Published: January 2006

The goal of this study was to elucidate the molecular mechanism by which type I IFN inhibits assembly and release of HIV-1 virions. Our study revealed that the IFN-induced ubiquitin-like protein ISG15 mimics the IFN effect and inhibits release of HIV-1 virions without having any effect on the synthesis of HIV-1 proteins in the cells. ISG15 expression specifically inhibited ubiquitination of Gag and Tsg101 and disrupted the interaction of the Gag L domain with Tsg101, but conjugation of ISG15 to Gag or Tsg101 was not detected. The inhibition of Gag-Tsg101 interaction was also detected in HIV-1 infected, IFN-treated cells. Elimination of ISG15 expression by small interfering RNA reversed the IFN-mediated inhibition of HIV-1 replication and release of virions. These results indicated a critical role for ISG15 in the IFN-mediated inhibition of late stages of HIV-1 assembly and release and pointed to a mechanism by which the innate antiviral response targets the cellular endosomal trafficking pathway used by HIV-1 to exit the cell. Identification of ISG15 as the critical component in IFN-mediated inhibition of HIV-1 release advances the understanding of the IFN-mediated inhibition of HIV-1 replication and uncovers a target for the anti HIV-1 therapy.
Stable and robust polymer nanotubes stretched from polymersomes
Authors: Reiner Joseph E. , Wells Jeffrey M. , Kishore Rani B. , Pfefferkorn Candace , Helmerson Kristian
Published: January 2006

We create long polymer nanotubes by directly pulling on the membrane of polymersomes using either optical tweezers or a micropipette. The polymersomes are composed of amphiphilic diblock copolymers, and the nanotubes formed have an aqueous core connected to the aqueous interior of the polymersome. We stabilize the pulled nanotubes by subsequent chemical cross-linking. The cross-linked nanotubes are extremely robust and can be moved to another medium for use elsewhere. We demonstrate the ability to form networks of polymer nanotubes and polymersomes by optical manipulation. The aqueous core of the polymer nanotubes together with their robust character makes them interesting candidates for nanofluidics and other applications in biotechnology.
Oviposition deterrents for the Mediterranean fruit fly, Ceratitis capitata (Diptera: Tephritidae) from fly faeces extracts
Authors: Arredondo J. , Díaz-Fleischer F.
Published: January 2006

After oviposition, females of the Mediterranean fruit fly Ceratitis capitata Wiedemann deposit a host-marking pheromone on the fruit surface that deters oviposition by conspecifics. Methanolic extracts of fruit fly faeces elicit a similar deterrent effect. The results of laboratory and field experiments using raw methanolic extracts of C. capitata faeces as an oviposition deterrent are reported. Laboratory bioassays revealed a significant positive relationship between concentration of faeces and the inhibition of oviposition responses by C. capitata. Treatment of halves of coffee bushes with methanolic extracts containing 0.1, 1.0 and 10 mg faeces ml−1 resulted in a significant reduction of infestation only at the highest concentration (P = 0.03). Treatment of blocks of coffee bushes with an extract of 10 mg faeces ml−1 resulted in an 84%reduction in infestation by C. capitata in sprayed plants and a 56% reduction in adjacent untreated coffee bushes surrounding treated plots, probably due to the deterrent effect of host-marking pheromone on fly oviposition. We conclude that faeces contain oviposition deterrent substances that effectively reduce fruit infestations by C. capitata, suggesting a clear potential for the use of this infochemical in integrated management programmes targeted at this pest.
Long-chainn−3 PUFA: plantv. marine sources
Authors: Williams Christine M. , Burdge Graham
Published: January 2006

Increasing recognition of the importance of the long-chainn−3 PUFA, EPA and DHA, to cardiovascular health, and in the case of DHA to normal neurological development in the fetus and the newborn, has focused greater attention on the dietary supply of these fatty acids. The reason for low intakes of EPA and DHA in most developed countries (0·1–0·5 g/d) is the low consumption of oily fish, the richest dietary source of these fatty acids. An important question is whether dietary intake of the precursorn−3 fatty acid, α-linolenic acid (αLNA), can provide sufficient amounts of tissue EPA and DHA by conversion through then−3 PUFA elongation–desaturation pathway. αLNA is present in marked amounts in plant sources, including green leafy vegetables and commonly-consumed oils such as rape-seed and soyabean oils, so that increased intake of this fatty acid would be easier to achieve than via increased fish consumption. However, αLNA-feeding studies and stable-isotope studies using αLNA, which have addressed the question of bioconversion of αLNA to EPA and DHA, have concluded that in adult men conversion to EPA is limited (approximately 8%) and conversion to DHA is extremely low (<0·1%). In women fractional conversion to DHA appears to be greater (9%), which may partly be a result of a lower rate of utilisation of αLNA for β-oxidation in women. However, up-regulation of the conversion of EPA to DHA has also been suggested, as a result of the actions of oestrogen on Δ6-desaturase, and may be of particular importance in maintaining adequate provision of DHA in pregnancy. The effect of oestrogen on DHA concentration in pregnant and lactating women awaits confirmation.
Prevalence and association of PCR ribotypes of Clostridium difficile isolated from symptomatic patients from Warsaw with macrolide-lincosamide-streptogramin B (MLSB) type resistance
Authors: Pituch Hanna , Brazier Jon S. , Obuch-Woszczatyński Piotr , Wultańska Dorota , Meisel-Mikołajczyk Felicja , Łuczak Mirosław
Published: January 2006

Isolates (79 in total) of Clostridium difficile obtained over a 2 year period from 785 patients suspected of having C. difficile-associated diarrhoea (CDAD) and being hospitalized in the University Hospital in Warsaw were characterized by toxigenicity profile and PCR ribotyping. Furthermore, their susceptibility to clindamycin and erythromycin was determined. Among the 79 C. difficile isolates, 35 were classified as A+B+, 1 as A+B+CDT+, 36 as A−B+ and 7 as A−B−. A total of 21 different PCR ribotypes was detected. Two main A+B+ strains circulated in our hospital: ribotype 014 and ribotype 046. Unexpectedly, the predominant PCR ribotype was type 017, a known A−B+ strain, and this accounted for about 45·5 % of all isolates cultured from patients with CDAD. Isolates belonging to PCR ribotype 017 were found in cases from epidemics of antibiotic-associated diarrhoea in the internal and surgery units. High-level resistance (MIC⩾256 mg l−1) to clindamycin and erythromycin was found in 39 (49 %) of the C. difficile isolates. Interestingly, 34 (94 %) of macrolide-lincosamide-streptogramin B (MLSB) type resistance strains did not produce toxin A, but produced toxin B and were A−B+ ribotype 017. Thirty-seven of the high-level resistance strains harboured the erythromycin-resistance methylase gene (ermB). C. difficile isolates (2/29) that had high-level clindamycin and erythromycin resistance, and belonged to PCR ribotype 046, were ermB negative. These investigations revealed that the predominant C. difficile strain isolated from symptomatic patients hospitalized in University Hospital in Warsaw was MLSB-positive clindamycin/erythromycin-resistant PCR ribotype 017.
Functional analysis of the competence transcription factor ComK of Bacillus subtilis by characterization of truncation variants
Authors: Susanna Kim A. , Fusetti Fabrizia , Thunnissen Andy-Mark W. H. , Hamoen Leendert W. , Kuipers Oscar P.
Published: January 2006

The competence transcription factor ComK is the master regulator of competence development in Bacillus subtilis. In the regulatory pathway, ComK is involved in different interactions: (i) protein–DNA interactions to stimulate transcription of ComK-dependent genes and (ii) protein–protein interactions, divided into interactions with other proteins and interactions between ComK proteins involving oligomerization. The fact that ComK displays different types of interactions suggests the presence of specific, distinct domains in the protein. This paper describes a search for functional domains, by constructing ComK truncation variants, which were tested for DNA binding, oligomerization and transcription activation. Truncations at the C-terminal end of ComK demonstrated the requirement of this part for transcription activation, but not for DNA binding. The C-terminal region is probably involved in oligomerization of ComK-dimers into tetramers. Surprisingly, a ComK truncation variant lacking 9 aa from the N-terminal end (ΔN9ComK) showed higher transcription activation than wild-type ComK, when expressed in Lactococcus lactis. However, in B. subtilis, transcription activation by ΔN9ComK was twofold lower than that by wild-type ComK, resulting from a five- to sixfold lower protein level of ComKΔN9. Thus, relatively, ΔN9ComK is more active in transcription activation than wild-type ComK. These results suggest that the presence of this N-terminal extension on ComK is a trade-off between high transcription activation and a thus far unidentified role in regulation of ComK.
Transcriptional and translational expression patterns associated with immobilized growth of Campylobacter jejuni
Authors: Sampathkumar Balamurugan , Napper Scott , Carrillo Catherine D. , Willson Philip , Taboada Eduardo , Nash John H. E. , Potter Andrew A. , Babiuk Lorne A. , Allan Brenda J.
Published: January 2006

AlthoughCampylobacter jejuniis a leading cause of food-borne illness, little is known about the mechanisms by which this pathogen mediates prolonged environmental survival or host cell virulence. Although these behaviours represent distinct phenotypes, they share a common requirement of an immobilized state. In order to understand the cellular mechanisms that facilitate a surface-associated lifestyle, transcriptional and translational expression profiles were determined for sessile and planktonicC. jejuni. These investigations indicate that the immobilized bacteria undergo a shift in cellular priorities away from metabolic, motility and protein synthesis capabilities towards emphasis on iron uptake, oxidative stress defence and membrane transport. This pattern of expression partially overlaps those reported for Campylobacter during host colonization, as well as for other species of bacteria involved in biofilms, highlighting common adaptive responses to the conserved challenges within each of these phenotypes. The adaptation of Campylobacter to immobilized growth may represent a quasi-differentiated state that functions as a foundation for further specialization towards phenotypes such as biofilm formation or host cell virulence.
Phenol/water extract of Treponema socranskii subsp. socranskii as an antagonist of Toll-like receptor 4 signalling
Authors: Lee Sung-Hoon , Kim Kack-Kyun , Rhyu In-Chul , Koh Sukhoon , Lee Dae-Sil , Choi Bong-Kyu
Published: January 2006

Treponema socranskii is one of the most frequently found oral spirochaetes in periodontitis and endodontic infections. LPS or glycolipids from bacteria are potent stimulators of innate immune and inflammatory systems. In this study the bioactivity of a phenol/water extract from T. socranskii subsp. socranskii (TSS-P) was analysed. TSS-P showed minimal endotoxicity and no inducing potential for proinflammatory cytokines (TNF-α and IL-8) or for intercellular adhesion molecule-1 (ICAM-1) in human monocyte cell line THP-1 cells and primary cultured human gingival fibroblasts. Rather, it inhibited ICAM-1 expression and IL-8 secretion from cells stimulated by the LPS of Escherichia coli and Actinobacillus actinomycetemcomitans, which are known to be Toll-like receptor 4 (TLR4) agonists. However, this antagonistic activity was not shown in cells stimulated by peptidoglycan or IL-1β. As its antagonistic mechanism, TSS-P blocked the binding of E. coli LPS to LPS-binding protein (LBP) and CD14, which are molecules involved in the recruitment of LPS to the cell membrane receptor complex TLR4–MD-2 for the intracellular signalling of LPS. TSS-P itself did not bind to MD-2 or THP-1 cells, but inhibited the binding of E. coli LPS to MD-2 or to the cells in the presence of serum (which could be replaced by recombinant human LBP and recombinant human CD14). The results suggest that TSS-P acts as an antagonist of TLR4 signalling by interfering with the functioning of LBP/CD14.
MfLIP1, a gene encoding an extracellular lipase of the lipid-dependent fungus Malassezia furfur
Authors: Brunke Sascha , Hube Bernhard
Published: January 2006

Malassezia furfur is a dimorphic fungus and a member of the normal cutaneous microflora of humans. However, it is also a facultative pathogen, associated with a wide range of skin diseases. One unusual feature of M. furfur is an absolute dependency on externally provided lipids which the fungus hydrolyses by lipolytic activity to release fatty acids necessary for both growth and pathogenicity. In this study, the cloning and characterization of the first gene encoding a secreted lipase of M. furfur possibly associated with this activity are reported. The gene, MfLIP1, shows high sequence similarity to other known extracellular lipases, but is not a member of a lipase gene family in M. furfur. MfLIP1 consists of 1464 bp, encoding a protein with a molecular mass of 54·3 kDa, a conserved lipase motif and an N-terminal signal peptide of 26 aa. By using a genomic library, two other genes were identified flanking MfLIP1, one of them encoding a putative secreted catalase, the other a putative amine oxidase. The cDNA of MfLIP1 was expressed in Pichia pastoris and the biochemical properties of the recombinant lipase were analysed. MfLip1 is most active at 40 °C and the pH optimum was found to be 5·8. The lipase hydrolysed lipids, such as Tweens, frequently used as the source of fatty acids in M. furfur media, and had minor esterase activity. Furthermore, the lipase is inhibited by different bivalent metal ions. This is the first molecular description of a secreted lipase from M. furfur.
Communal spawning of brook and river lampreys, Lampetra planeri Bloch and Lampetra fluviatilis L.
Authors: Huggins R. J. , Thompson Anne
Published: January 2006

A description is given of the spawning of brook lampreys Lampetra planed Bloch and unusually small river lampreys Lampetra fluviatilis L. in the same redd. No successful interspecific copulation was observed, although the possibility of accidental cross fertilization cannot be excluded.
Diel changes in invertebrate drift and the food of trout Salmo trutta L.
Authors: Elliott J. M.
Published: January 2006

Diel changes in the stomach contents of 0+, 1 + and 2+ or older trout were compared with diel changes in invertebrate drift. Peaks in mean numbers and biomass of invertebrates per fish stomach occurred in the midday (10.00 to 14.00 h) and evening (18.00 to 22.00 h) samples. The major peak was usually in the evening sample, but the major peak in biomass was in the midday sample for 2+ fish. There was a good correlation between diel changes of benthic invertebrates in the diet and drift, with the major peak in the evening. A similar relationship did not always exist for emerging and terrestrial invertebrates. Known non‐drifting benthic invertebrates (e.g. large caddis larvae) were excluded from the comparisons, and were only taken by 2+ or older trout in which they contributed a large biomass to the day food.
Some notes on an ambivalent behaviour of the Greenland halibut Reinhardtius hippoglossoides (Walb.) Pisces: Pleuronectiformes
Authors: de Groot S. J.
Published: January 2006

The ambivalent behaviour of the Greenland halibut with respect to its swimming position is discussed. The conclusion is that, when swimming close to the bottom, it swims the way all flatfishes do, viz. horizontally; when it swims freely, however, it adopts a vertical position.
Effect of temperature on rate of passage of food through the alimentary canal of the plaice Pleuronectes platessa L.
Authors: Edwards D. J.
Published: January 2006

The relation between temperature and rate of passage of pieces of Arenicola marina through the gut of Pleuronectes platessa was investigated by an X‐ray method, using barium sulphate as a contrast medium. Time in hours taken for stomach evacuation and travel of gut contents to the rectum plotted against temperature gave negative exponential curves. Plotted logarithmically the relationships were linear.
Intestinal helminths in rainbow trout, Salmo gairdneri (Richardson): Absence of effect on nutrient absorption and fish growth
Authors: Ingham L. , Arme C.
Published: January 2006

Rainbow trout. Salmo gairdneri (Richardson), from a fish farm, were found to be infected with two cestodes, Eubothrium crassum and Proteocephalus sp. Observations are described which suggest that these parasites have no effect on fish growth or on the intestinal absorption of L‐leucine and D‐glucose.
Histochemical analysis of mucous cells in the epidermis of brown trout Salmo trutta L.
Authors: Harris J. E. , Watson Aileen , Hunt S.
Published: January 2006

A histochemical study of the epidermal mucous cells of brown trout revealed that they contained both neutral and acidic mucosubstances which were diastase‐resistant, PAS‐reactive. Neuraminidase treatment, methylation and combined staining procedures suggested that the acidic nature of the mucins was due mainly to the presence of sialic‐acid containing glycoprotein. These results augment data derived from biochemical analyses of the epidermal mucus of salmonid fish.
Changes in urine flow rate and haematocrit value of rainbow trout Salmo gairdneri (Richardson) exposed to hypoxia
Authors: Swift D. J. , Lloyd R.
Published: January 2006

Measurements were made of urine flow rates and haematocrit of rainbow trout exposed to hypoxic conditions (3 mg O2/1) for 3, 4, 5 or 24 h. Elevated urine flow rates during short time exposure and concomitant increased haematocrit, followed by lower than normal urine flow rates on return to normal aerated water, suggest that haemoconcentration is the initial response to anoxia. Results from longer exposure to hypoxic conditions suggest that the initial period of haemoconcentration is followed by a period of water retention and a return of the blood volume to normal; the sustained elevated haematocrit is thought to be derived from release of erythrocytes from storage organs. During prolonged exposure rainbow trout also become more permeable to water.
Aspects of the biology of Tilapia mossambica Peters (Pisces: Cichlidae) in a natural freshwater lake (Lake Sibaya, South Africa)
Authors: Bruton M. N. , Boltt R. E.
Published: January 2006

An account is given of some aspects of the biology of Tilapia mossambica Peters in Lake Sibaya, South Africa. Lake Sibaya is a warm shallow coastal lake with extensive shallow terraces in the littoral which shelve abruptly into underwater valleys. The substrate is predominantly sandy. Aquatic macrophytes are generally restricted to water 1–7 m deep. Adult fishes (over 8 cm standard length) are usually absent from water deeper than 12m and shallower than 0–5 m, whereas juveniles may occur at all depths, and fry only in very shallow water.

T. mossambica inhabits the littoral and sublittoral in the warm and transition periods (August–April) but moves into deep water in the cool season (May–July). Exposed and sheltered areas are utilized for different purposes by adult fishes, the former for nesting, and latter for feeding and mouth–brooding. Habitat selection by males is governed by the availability of suitable nest sites. Nests are most common in sparsely–vegetated sheltered areas. Brooding females preferred sheltered littoral areas but ventured onto the terrace to release the fry. Juveniles and fry inhabited exposed shores with a temperature gradient which reversed diurnally. The breeding, shoaling and feeding behaviour is described. The biology of T. mossambica in Lake Sibaya is compared to data on the same, and similar species, in other systems. The utilization of available resources in the lake by T. mossambica is discussed and reference is made to the importance of the retention of generalized characters for the successful habitation of the cyclically–renewed habitat of the littoral.
Studies on the swimming musculature of the rainbow trout.
Authors: Johnston I. A.
Published: January 2006

Some aspects of anaerobic metabolism in the red and white (mosaic) muscles of the rainbow trout have been studied during environmental hypoxia. Evidence has been obtained for the operation of anaerobic pathways other than glycolysis, especially in the superficial red muscle. L‐alanine and succinate were observed to accumulate as anaerobic endproducts in this tissue. Anaerobic glycolysis also increased significantly during hypoxia, but only in the white muscle. Glycogen stores were depleted by 70–85 % in both red and white muscle. Glucose increased in concentration in the red but not in the white muscle. In an attempt to understand some of the species differences in the susceptibility of fish to hypoxia the results of the present study have been compared with an earlier study on the carp (Johnston, 1975). The effect of death from hypoxia on the levels of muscle metabolites has also been studied.