bluuebunny/crossref_metadata_2025_split huggingface dataset:
Multisystem involvement in a patient due to accumulation of amylopectin‐like material with diminished branching enzyme activity
Authors: Eminoglu T. F. , Tumer L. , Okur I. , Olgunturk R. , Hasanoglu A. , Gonul I. I. , Dalgic B. 
Published: April 2008

SummaryWe report a 13‐year‐old boy with multisystem involvement secondary to accumulation of amylopectin‐like material. He was born to consanguineous parents at full term without any complications and his maternal perinatal history was uneventful. His parents were cousins. He had normal growth and development except for his weight. His sister died from an unexplained cardiomyopathy at the age of 8 years. Our patient's initial symptom was severe heart failure. Since he also had a complaint of muscle weakness, electromyography was performed which showed muscle involvement. The diagnosis was suggested by tissue biopsy of skeletal muscle showing intracellular, basophilic, diastase‐resistant, periodic acid–Schiff‐positive inclusion bodies and was confirmed by the presence of a completed branching enzyme deficiency. Similar intracytoplasmic inclusion‐like bodies were also found in liver biopsy, but very few in number compared with the skeletal muscle. The patient died from an intercurrent infection. Postmortem endomyocardial biopsy revealed the same intracytoplasmic inclusions as described above affecting almost all myocardial cells. Ultrastructural examination of liver biopsy was nondiagnostic; however, myocardium showed prominent, large, intracytoplasmic deposits. Glycogen branching enzyme gene sequence was normal, andthus classical branching enzyme deficiency was excluded. Our patient represents the first molecular studyperformed on a patient in whom there was multiplesystem involvement secondary to accumulation of amylopectin‐like material. We suggest that this is an as yet undefined and different phenotype of glycogen storage disease associated with multisystemic involvement.
Pathogenesis of CNS involvement in disorders of amino and organic acid metabolism
Authors: Kölker S. , Sauer S. W. , Hoffmann G. F. , Müller I. , Morath M. A. , Okun J. G. 
Published: April 2008

SummaryInherited disorders of amino and organic acid metabolism have a high cumulative frequency, and despite heterogeneous aetiology and varying clinical presentation, the manifestation of neurological disease is common. It has been demonstrated for some of these diseases that accumulating pathological metabolites are directly involved in the manifestation of neurological disease. Various pathomechanisms have been suggested in different in vitro and in vivo models including an impairment of brain energy metabolism, an imbalance of excitatory and inhibitory neurotransmission, altered transport across the blood–brain barrier and between glial cells and neurons, impairment of myelination and disturbed neuronal efflux of metabolic water. This review summarizes recent knowledge on pathomechanisms involved in phenylketonuria, glutaric aciduria type I, succinic semialdehyde dehydrogenase deficiency and aspartoacylase deficiency with examples, highlighting general as well as disease‐specific concepts and their putative impact on treatment.
Flow–orbit equivalence for minimal Cantor systems
Authors: KOSEK WOJCIECH , ORMES NICHOLAS , RUDOLPH DANIEL J. 
Published: April 2008

AbstractThis paper is about flow–orbit equivalence, a topological analog of even Kakutani equivalence. In addition to establishing many basic facts about this relation, we characterize the conjugacies of induced systems that can be extended to a flow–orbit equivalence. We also describe the relationship between flow–orbit equivalence and a distortion function of an orbit equivalence. We show that, if the distortion of an orbit equivalence is zero, then it is in fact a flow–orbit equivalence, and that the converse is true up to a conjugation by an element of the full group.
The Effect of Knowledge of Results on Learning and Performance IV. The Direction of the Error in Very Simple Skills
Authors: Dees Valerie , Grindley G. C. 
Published: April 2008

Re-examination of the data discussed in previous papers of this series shows a greater tendency toward “overshooting” when the time interval between trials, in some of the experiments, is short than when it is longer. The subject tends to make a bigger movement or exert more pressure with short intervals. This seems to be true with or without visual knowledge of results. On the other hand, with the experiments in which the task was to press a key for a given short interval, the effect was not conclusively shown. A hypothesis is put forward to explain these results in terms of proprioceptive adaptation.
Transfer of Training and Skill Assumptions in Tracking Tasks
Authors: Gibbs C. B. 
Published: April 2008

Matched groups of subjects were used to test the learning and transfer effects that follow changes in the display, the muscular reactions and the directional relationship between stimulus and response in a tracking task. Two arrangements were compared in the relationship studies: one arrangement of the stimuli and reactions was similar, and the other was opposed to that used in many every-day skills. The familiar arrangement was easier to learn. There was high positive transfer from the unfamiliar to the familiar, and little transfer from the familiar to the unfamiliar. The physical dimensions of the display were varied to give two tasks with different stimuli. The initial learning times were equal for both tasks, and the transfer between them was high, positive, and equal. Two further tasks varied in the extent, speed and force of the required muscular movements. One task proved more difficult to learn initially, and there was greater transfer from the difficult to the easy task than from the easy to the difficult. A further experiment tested the effects of changing the difficulty of a tracking course, and it was found that learning was more rapid on the more difficult course. A difference in difficulty between two tasks, therefore, determined both the amount of transfer between them and the rate of learning the tasks. New measures were developed to test the transfer between tasks of unequal content, and the effect of such inequalities upon the rate of learning. The findings are discussed, as are their possible implications for transfer measurement and their bearing upon existing theories of transfer.
Learning of a Serial Task by Different Age Groups
Authors: Kay H. 
Published: April 2008

In a serial learning task given to subjects whose ages ranged from twenty to seventy, a decline in performance showed itself as a loss of speed in the thirties, a loss of accuracy in the forties, and a marked loss of both speed and accuracy in the fifties and sixties. Experiments on recall and reorganization of material verified that older subjects were forgetting more quickly than younger, but that one of their main difficulties was an inability to substitute new reponses in an already mentally formed sequence. Older subjects were not introducing new learning features but accentuating normal tendencies, such as the repetition of the same error at the same serial position. In so far as subjects learned their repeated errors, final learning involved unlearning, with the often observable phenomenon of the previously learned error serving as the cue for the more recently learned correct response. In terms of schematic concepts of mental functions, serial learning difficulty was not so much the formation of a general “scheme” but its subsequent amendment. Preformed habits influenced both the learning procedure and its results, particularly in the constraints and expectancies which the making of one serial response had upon another.
A Note on Immediate Memory
Authors: Zangwill O. L. 
Published: April 2008

In the course of an extensive inquiry into verbal memory processes (Gomulicki, 1952), some data were collected which appear to throw fresh light on the nature of immediate memory. In particular, it was found that many of the varieties of constructive change defined by Bartlett (1932) in connexion with long-term recall could be traced in the immediate reproduction of passages so brief as barely to exceed the memory-span. As changes of this nature do not appear to have been previously described in connexion with short-term recall some illustrative extracts from our material will be given. A full analysis along somewhat different lines has been presented elsewhere (Gomulicki, 1952). In Gomulicki's experiments, narratives varying in length between 15 and 200 words were read aloud to 50 subjects, who were required to reproduce each passage orally as accurately as possible immediately after it had been read. The subjects (of whom half were women) were University graduates and undergraduates aged between 20 and 30. All subjects were tested individually and their reproductions recorded verbatim. In the present note, we shall be concerned only with the results given in the case of two passages, respectively 22 and 44 words in length. Despite their brevity, striking and consistent changes were observed in the reproductions which merit brief analysis.
Studies in Olfactory Acuity. I: Measurement of Olfactory Thresholds in the Rat
Authors: Eayrs J. T. , Moulton D. G. 
Published: April 2008

The capacity of the rat to respond to olfactory cues has been studied in three experimental situations: (i) an elevated Y-maze, (ii) a rectangular choice apparatus in which extraneous environmental cues were present, and (iii) a circular choice apparatus from which such cues were either excluded or randomized. No rat showed any sign of learning on the Y-maze even after extensive training and the application of electric shock as deterrent for error. Some rats succeeded in performing an olfactory discrimination in the rectangular choice apparatus but learning was retarded, and in some cases inhibited, by the counter-influence of non-olfactory factors. All rats readily learned an identical discrimination when shielded from such cues in the circular choice apparatus which provides a satisfactory medium both for training and for the study of olfactory acuity. Differences in the behaviour of rats on these three types of apparatus suggest first, that the formation of learned habits based on olfaction depends on the close contiguity of stimulus and reward, and second that the sense of smell possesses weak orientating properties by comparison with those exerted by other modalities. The findings thus help to explain differences in the ease or difficulty uith which olfactory discriminations have been established by earlier workers.
Shorter Articles and Notes two more Visual Theorems
Authors: Brindley G. S. 
Published: April 2008

The basis of the widely held opinion that the spectral sensitivities of the mechanisms determining trichromacy must be linearly related to the spectral mixture functions is examined, and a proof from simple assumptions is stated. It is proved also that, on the van der Velden hypothesis of independent sensitive units, a spatial or temporal summation law of the form AIn = c implies a frequency-of-seeing curve of the form Q = I— e−KIn ( Q = probability of detecting a stimulus of size A and intensity I; n, c and K are constants).
The Effect of Temporal Separation and Prior Habituation upon Preconditioning
Authors: Howarth Edgar 
Published: April 2008

The effect of temporal separation and prior habituation was tested by measuring response transfer in a shuttle box following different initial treatments. Thirty hooded rats were split into three main groups and six sub-groups. One main group was preconditioned with concurrent light and sound stimuli, another with temporally spaced stimuli, and a control group had no preconditioning. Half of each main group was permitted to explore the apparatus before preconditioning on the supposition that this might reduce emotionality and hence reduce startle or attention responses. It was found that concurrent stimulation produced significantly greater response transfer than in controls, whereas preconditioning with spaced stimuli did not. Prior habituation produced no significant effect on the amount of response transfer.
p38 mitogen‐activated protein kinase inhibitor LY2228820 enhances bortezomib‐induced cytotoxicity and inhibits osteoclastogenesis in multiple myeloma; therapeutic implications
Authors: Ishitsuka Kenji , Hideshima Teru , Neri Paola , Vallet Sonia , Shiraishi Norihiko , Okawa Yutaka , Shen Zhenxin , Raje Noopur , Kiziltepe Tanyel , Ocio Enrique M. , Chauhan Dharminder , Tassone Pierfrancesco , Munshi Nikhil , Campbell Robert M. , Dios Alfonso De , Shih Chuan , Starling James J. , Tamura Kazuo , Anderson Kenneth C. 
Published: April 2008

SummaryThe interaction between multiple myeloma (MM) cells and the bone marrow (BM) microenvironment induces proliferation and survival of MM cells, as well as osteoclastogenesis. This study investigated the therapeutic potential of novel p38 mitogen‐activated protein kinase (p38MAPK) inhibitor LY2228820 (LY) in MM. Although cytotoxicity against MM cell lines was modest, LY significantly enhanced the toxicity of bortezomib by down‐regulating bortezomib‐induced heat shock protein 27 phosphorylation. LY inhibited interleukin‐6 secretion from long term cultured‐BM stromal cells and BM mononuclear cells (BMMNCs) derived from MM patients in remission. LY also inhibited macrophage inflammatory protein‐1α secretion from patient MM cells and BMMNCs as well as normal CD14 positive osteoclast precursor cells. Moreover, LY significantly inhibitedin vitroosteoclastogenesis from CD14 positive cells induced by macrophage‐colony stimulating factor and soluble receptor activator of nuclear factor‐κB ligand. Finally, LY also inhibitedin vivoosteoclatogenesis in a severe combined immunodeficiency mouse model of human MM. These results suggest that LY represents a promising novel targeted approach to improve MM patient outcome both by enhancing the effect of bortezomib and by reducing osteoskeletal events.
Importance of murine Vδ1+γδ T cells expressing interferon‐γ and interleukin‐17A in innate protection against Listeria monocytogenes infection
Authors: Hamada Satoru , Umemura Masayuki , Shiono Takeru , Hara Hiromitsu , Kishihara Kenji , Tanaka Kensho , Mayuzumi Hirokazu , Ohta Takao , Matsuzaki Goro 
Published: April 2008

SummaryMurine γδ T cells participate in the innate immune response against infection by an intracellular pathogen Listeria monocytogenes. Vδ1+γδ T cells coexpressing Vγ6 are a major γδ T‐cell subpopulation induced at an early stage of L. monocytogenes infection in the livers of infected mice. To investigate the protective role of the Vγ6/Vδ1+γδ T cells against L. monocytogenes infection, Vδ1 gene‐deficient (Vδ1−/−) mice were analysed because these mice selectively lacked a Vγ6/Vδ1+γδ T‐cell subpopulation in the L. monocytogenes‐infected liver. The Vδ1−/− mice showed increased bacterial burden in the liver and spleen, and decreased survival rate at an early stage of L. monocytogenes infection when compared to wild‐type mice. Histological examination showed abscess‐like lesions and unorganized distribution of macrophages in the liver of the Vδ1−/− mice but not in the wild‐type mice after L. monocytogenes infection. The Vγ6/Vδ1+γδ T cells produced interferon‐γ and interleukin‐17A. All the results suggest that murine Vγ6/Vδ1+γδ T cells control the innate protective response against L. monocytogenes infection through production of the proinflammatory cytokines interferon‐γ and interleukin‐17A in the infected liver.
Cloning and molecular characterization of two invertebrate‐type lysozymes from Anopheles gambiae
Authors: Paskewitz S. M. , Li B. , Kajla M. K. 
Published: April 2008

AbstractWe sequenced and characterized two novel invertebrate‐type lysozymes from the mosquito Anopheles gambiae. Alignment and phylogenetic analysis of these and a number of related insect proteins identified through bioinformatics strategies showed a high degree of conservation of this protein family throughout the Class Insecta. Expression profiles were examined for the two mosquito genes through semiquantitative and real‐time PCR analysis. Lys i‐1 transcripts were found in adult females in the fat body and Malpighian tubules, whereas Lys i‐2 was detected only in fat bodies. Blood‐feeding resulted in significantly increased transcript abundance for both genes in the midguts. Neither gene was upregulated following bacterial challenge.
Trying times with a turtle*
Authors: Parry M. T. 
Published: April 2008

AbstractThis paper is an edited and much‐abridged diary of the day‐to‐day problems encountered by the author in her attempt to set up a school investigation. This was a controlled experiment involving the use of floor and screen turtles with two groups of fourth year primary school children. Apart from the educational, psychological and administrative problems involved in the implementation of such an experiment, the practical difficulties were many.
Report to the Alvey Directorate on a short survey of expert systems in UK business*
Authors: D'Agapeyeff A. O 
Published: April 2008

AbstractSimpler expert systems are practical and being implemented now by self‐taught teams, with little risk at relatively low‐cost, to produce modest but unusual gains. It is necessary to correct the impression, much heralded hitherto, that expert systems are inherently complex, risky and demanding. This impression is a handicap to competitive developments in the supply and usage of advanced information technology.
Information Technology in Education Research Programme 1988–93
Authors: Lewis R. 
Published: April 2008

Abstract Following a 3‐year pilot programme, the Economic and Social Research Council (ESRC) undertook to play a further major role in the evolution of new ways in which technology could contribute to learning by supporting some of the necessary basic research. It was accepted that the uncertain pragmatics of early work on classroom technologies required more clearly defined studies. Cognitive science, which promised to provide the basic concepts for work on teaching and learning, was seen to be achieving some maturity. Nevertheless, current work remained somewhat scattered. Whilst diversity of approach remains undoubtedly important when new problems are to be solved, an investment was required in broadly managed programmes across this multidisciplinary field to promote the development of ‘critical mass’ and the emergence of directions for change.A Coordinating Centre, a Programme Evaluation Unit and three Research Centres have been approved for work over 3 years (1988‐91) with support from ESRC of over Ł1M as part of the research initiatives programme of the Human Behaviour and Development Research Development Group.
The independent policy evaluation
Authors: MacDonald B. , Stronach I. 
Published: April 2008

Abstract This article is based on the proposal for evaluation submitted to the ESRC by the authors. The proposal offered an independent policy evaluation which would help people outside the Programme to know and judge what is going on, and those taking part in the programme to take into better account the impact on others of their activities and findings. The thrust of the proposal is formative, and focuses on the communication of research knowledge both within the programme, and between the programme and various interest groups and policy‐making bodies. The major concern of the evaluation is to understand and represent — through formative evaluation — the potential impact of the Programme on its intended audiences and users. This implies a focus on the research activities themselves (in so far as these emphases are separable). The relationship between the ESRC InTER Programme and the policy evaluation is an independent one. That is to say, the evaluation is sponsored by the ESRC, but is committed to independent internal and public reporting.The methods favoured by this evaluation are known by the term ‘naturalistic’, so‐called because they are like the ways in which people generally come to understand social life — looking, listening, and talking to people whose actions, experience and reasoning we have to represent and report in a useful form, and the article ends with a brief methodological justification for such an approach to policy evaluation.
Coherence & autonomy: issues in the evaluation of an open learning project
Authors: Saunders M. 
Published: April 2008

Abstract  This paper will elaborate some of the key areas of interest in the evaluation of the JITOL, a project funded under the European Community DELTA initiative. The purpose of the JITOL (Just in Time Open Learning) project is to develop and evaluate a learning environment for the professional development of individuals. The environment is based upon information technology consisting of telematic network linking and making available computer resources, including data and knowledge bases, and using conferencing and communications software to facilitate access to human tutors and co‐learners.The three domains in which testing and validation of the JITOL model will take place represent areas in which real user/learner interest is already evident. From the point of view of evaluation, the JITOL project is characterized in such a way that it raises some particular challenges.
Investigation of the pattern of diversity of hepatitis C virus in relation to times of transmission
Authors: Simmonds P. , Smith D. B. 
Published: April 2008

Summary. The rate of sequence change of HCV in vivo was used to date the spread of HCV genotype 1b in European, USA and Japanese populations. Silent substitution rates of 0.0011 and 0.0017 substitutions per site per year were observed in the NS5 and E1 regions by sequence comparisons from a cohort of individuals infected from a common source of infection 17 years previously. Mean silent substitution frequencies of 0.169 and 0.224 in NS5 and E1, respectively, were observed amongst type 1b variants infecting epidemiologically unrelated individuals from several countries. This predicted a time of divergence from a common ancestor of 64–69 years. The absence of country‐specific groupings by phylogenetic analysis of these sequences suggested that the spread of this genotype occurred on a worldwide basis at a similar time. Dates for the spread of other genotypes varied from around 80 years (type 2a) to 54 years (type 3a), suggesting that different variants spread into communities at different times this century.By extrapolating the silent substitution rate, the time of divergence of type 1a from 1b can be estimated at 200–300 years, but even this is likely to be an underestimate of the true time due to inequalities on the transition and transversion ratios, and the greater frequency of G ⊼ A transitions, compared with C ⊼ U. which are not considered in the current analysis. Thus, the diversity of the globally distributed genotypes such as type 1b and 3a suggests a relatively recent origin for HCV in Western countries and Japan, and contrast with the much greater diversity of specific genotypes in Central Africa (type 4) and South East Asia (type 6). These data may assist in understanding the global epidemiology of HCV and the mechanisms by which it has spread.