bluuebunny/crossref_metadata_2025 huggingface dataset:
Mediated Geographies: Critical Pedagogy and Geographic Education
Authors: Lukinbeal Chris , Kennedy Christina B , Jones John Paul , Finn John , Woodward Keith , Nelson David A , Grant Zane Austin , Antonopolis Nicole , Palos Ari , Atkinson-Palombo Carol
Published: August 2007

Motivated by a need to engage students in the critical evaluation of visual information, and by a desire to teach students how to use digital technologies as a way of exploring and expressing geographical constructs and processes, the geography departments at Arizona's three universities sought and received funding from the Arizona Board of Regents for learner-centered curricular development organized around the theme of "Mediated Geographies." In this paper, we explore how critical pedagogy and learner-centered education strategies were used to engage students in semester-long documentary and photo essay projects. Some of the student projects discussed in this essay are posted for viewing at the project Web site: http://geography.asu.edu/lukinbeal/mediated.html. This project was funded by the Arizona Board of Regents' (ABOR) Learner Centered Education Grant Program. For more information about ABOR's program, see http://www.abor.asu.edu/4_special_programs/lce/index_lce.html.
The Changing Faces of Skagway, Alaska: A Story So Far
Authors: Moreno Christopher M
Published: August 2007

In this paper, I explore the multiple trajectories and relational politics from which Skagway, Alaska, has "become the new." I engage Skagway through a "language of movement." I tell "a story so far" about Skagway, Alaska, as a "place in motion." The paper unfolds through an ethno-historic narrative of the multiple lines of social and economic differentiation, processes of change, and numerous face-lifts Skagway has undergone since the Klondike Gold Rush on through to today's connectivity with the global cruise tourism industry.
The Changing Cultural Landscape of El Paso de Robles
Authors: Peters Gary L
Published: August 2007

In and around the city of El Paso de Robles, the cultural landscape is changing rapidly. Two major causes of these landscape changes are population growth and the shift from cattle ranching to viticulture in the surrounding area. This paper focuses first on population growth and some of its impacts on the cultural landscape, then on the rise of viticulture and landscape features associated with it. Whereas most of the landscape changes driven by population growth are typical of places almost everywhere, those created by the upsurge in viticulture and the wine tourism associated with it are often unique and give the city and its environs a distinctive character and sense of place.
Urban Expansion in Oaxaca, Mexico: Research on the Fringe
Authors: Smith Marissa
Published: August 2007

This research examines the land use change on the urban edge of Oaxaca, Mexico, and the associated environmental and social consequences of this transformation in the peri-urban community and municipality of San Jacinto Amilpas (SJA). The city of Oaxaca is expected to grow from a current population of approximately half a million to over a million by 2010. The dramatic growth and subsequent environmental change embodied in the process of urbanization is altering the economic, cultural and political face of SJA. Interviews with community members indicate that these changes have resulted in increased crime and conflict, amplified class divisions, and a population increasingly focused on individual pursuits and new sources of livelihood which are slowly eroding traditional community cohesiveness.
DVD Reviews
Authors: Bould Mark , Evers Pamela S , Kreul James
Published: August 2007

Sweet Sweetback's Baadasssss Song (1971)

Baadasssss! (aka How To Get the Man's Foot Outta Your Ass) (2003)

Personal angst as driving force

Divan (2003)

Prom Night in Kansas City (2002)

Film ist. [112] DVD Edition (2004)

Films from a Dark Room (2004)
Festivals
Authors: Lumholdt Jan
Published: August 2007

Some tales of the expected: Internationale Filmfestspiele Berlin, 818 February 2007
Plastid division in an evolutionary context
Authors: Tveitaskog Astrid E. , Maple Jodi , Møller Simon G.
Published: August 2007

Plastids are derived from free-living cyanobacteria that were engulfed by eukaryotic host cells through the process of endosymbiosis and, like their cyanobacterial ancestors, divide by binary fission. Over the last decade the continued identification and functional analysis of plastid division components, coupled with ever-increasing genomic resources, have yielded insights into the origins and evolution of the plastid division mechanism in higher plants. Here we review the current understanding of the evolution of the chloroplast division proteins and present a model of how the machinery has developed to execute plastid division in Arabidopsis.
Variability of the mitochondrial genome in mammals at the inter-species/intra-species boundary
Authors: Santamaria Monica , Lanave Cecilia , Vicario Saverio , Saccone Cecilia
Published: August 2007

Genomic variations represent the molecular basis of the biodiversity of living organisms on which selection operates to generate evolution. In eukaryotes, genomic variability can be experienced in both nuclear and organellar, i.e. mitochondrial and plastid (where present), genomes, which can follow completely different evolution pathways, as revealed by comparative genomics analyses. In Metazoa, for which a substantial number of complete genome sequences are available (nuclear, but mainly mitochondrial), we are just starting to grasp the selective pressures operating on some basic features of the genome as a whole. In this brief review, we discuss the variability of the mitochondrial metazoan genome, with particular reference to mitochondrial DNA in mammals. In light of the recent assumption that a small segment of mitochondrial DNA may be used, particularly in Metazoa, as a species marker, some data on mitochondrial gene variability at the inter-species/intra-species boundary are reported. Intra-species variability has been evaluated in four mammalian species, Homo sapiens, Bos taurus, Sus scrofa and Canis familiaris, whereas the relationship between intra- and inter-species variability has been investigated in Bos taurus and Bos indicus.
‘Everyday language’ in emigrant letters and its implications for language historiography – the German case
Authors: Elspaß Stephan
Published: August 2007

The mass literacy drives of the 19thcentury have proved to be a landmark in German language history, as for the first time the majority of the people in the German-speaking countries were able to participate in the culture of writing. The full impact of the spread of writing among the lower social classes on language variation and change has, however, not yet been recognised in language historiography. With examples from grammar and spelling in private emigrant letters, the present article strongly argues for an alternative approach to language historiography, using such texts as a starting-point for a ‘language history from below’.
Writing and ‘the Standard’: England, 1795–1834
Authors: Fairman Tony
Published: August 2007

This article gives a detailed description and discussion of seven applications for poor relief, as found in the archives of English County Record Offices. All letters were written by or for poor writers between 1795 and 1834. As the variety used in these letters is not ‘Standard’, nor ‘dialect’, the second part of the article is devoted to the question of how this variety relates (or does not) to traditional linguistic views on standardisation. Contrary to the prevailing ‘standard language ideology’, it will be claimed that the present letters can only be interpreted adequately through a linguistic theory ‘which treats all varieties of English equally and discards none’.

… in writing the history of powerless people, drawing on conventional, published sources is far from enough.

Adam Hochschild (2002: 104)
Double diglossia – lower class writing in 19th-century Finland
Authors: Nordlund Taru
Published: August 2007

This paper analyses a number of typical features of lower-class writing in 19th-century Finland. The data consists of business letters written by local traders and farmers. These letters are compared to letters by Forest Finns, a group of early Finnish migrants to Scandinavia. The business letters are marked by three distinct influences: the older literary tradition, local dialects and the ‘rising literary genres’ of the 19thcentury. On the basis of the morpho-phonological level of the corpus texts, it will be argued that the lower-class writers intended to write a standard language, not a dialect.
Piezo-actuated zona-drilling improves the fertilisation of ops vitrified mouse oocytes
Authors: Meng Qinggang , Li Xiuwei , Wu Tongyi , Dinnyés A. , Zhu Shien
Published: August 2007

The present study was designed to investigate fertilisation of open pulled straw (OPS) vitrified mouse oocytes drilled with piezo-micromanipulation method and their subsequent
in vitro
and
in vivo
developmental capacity. Ovulated mouse oocytes were vitrified using the OPS method. After warming, the zona pellucida of a group of vitrified-warmed oocytes was drilled by piezo-micro-manipulation. Groups of (a) vitrified, (b) vitrified/drilled and (c) fresh control oocytes were fertilised
in vitro
. The fertilisation rate of vitrified-warmed oocytes was significantly lower than that of fresh oocytes (45.0 ± 12.6% vs. 85.2 ± 6.8%, P < 0.05), and was significantly improved by zona-drilling (85.4 ± 7.3%). However, blastocyst formation rates of the vitrified and vitrified/drilled groups were significantly lower than those of the fresh controls (65.7 ± 7.0% and 66.4 ± 2.5% vs. 86.6 ± 4.3%, respectively, P < 0.05). The cell number of blastocysts from the vitrified/drilled or the vitrified group was not different from that of the controls. Embryo transfer resulted in pregnancy in all three groups, but the rate of development to term was lower in the vitrified/drilled or vitrified groups than in the controls (16.6 ± 0.7% or 36.0 ± 2.4% vs. 51.3 ± 2.9%, respectively). Our results demonstrated that zona-drilling with piezo-micromanipulation could improve fertilisation in OPS vitrified mouse oocytes but did not increase the overall number of vitrified oocytes developing to term.
Dietary supplementation of obese children with 1000 mg alpha-linolenic acid per day: a placebo-controlled double blind study
Authors: Lohner Szimonetta , Marosvölgyi Tamás , Burus István , Schmidt János , Molnár Dénes , Decsi Tamás
Published: August 2007

Az omega-3 zsírsav fogyasztásának növelése kedvező hatású lehet a fokozott cardiovascularis kockázatú személyek, köztük az elhízottak számára. A kedvező hatások létrejöttének feltétele az omega-3 zsírsavaknak a szervezet plazmalipideibe történő beépülése, azonban a gyermekkorban ehhez szükséges szupplementációs dózisra vonatkozóan igen kevés adat áll rendelkezésre. A jelen vizsgálatunk célja napi 1000 mg alfa-linolénsavval kiegészített étrend a plazmalipidek zsírsavösszetételére kifejtett hatásának vizsgálata volt elhízott gyermekekben.
Személyek és módszerek:
Ebben a placebóval kontrollált, keresztező önkontrollos, 2 × 6 hétig tartó vizsgálatban a részt vevő 9 elhízott gyermek (életkor: 13,1 [±2,5] év, testtömegindex: 31,2 [±6,2] kg/m
2
, medián [IQR]) étrendjébe speciális takarmánnyal etetett tyúkoktól származó napi 1 tojást és 50 g csirkehúst építettünk be, amivel napi 1000 mg alfa-linolénsav bevitelét tudtuk biztosítani. A plazmalipidek zsírsavösszetételét nagy felbontóképességű kapilláris gáz-folyadék kromatográfiával határoztuk meg.
Eredmények:
Az alfa-linolénsav-értékek emelkedő tendenciája volt megfigyelhető az alfa-linolénsavval történő szupplementációt követően a foszfolipid-, a triacil-glicerin- és a szterol-észter-frakcióban egyaránt, azonban egyik frakcióban sem volt a változás szignifikáns. A nem észterifikált zsírsavfrakcióban az alfa-linolénsav értékei szignifikánsan emelkedtek (0,11 [0,08] szemben 0,14 [0,20], tömeg%,
p
< 0,05), jelezve az alfa-linolénsav plazmalipidekben történő feldúsulásának a kezdetét.
Következtetés:
Az elhízott gyermekekben az étrend 6 héten keresztül napi 1000 mg alfa-linolénsavval történő kiegészítése csak a plazma nem észterifikált zsírsavaiban növelte szignifikánsan az omega-3 zsírsavak arányát, az észterifikált frakciókban nem volt szignifikáns hatás. Elhízott gyermekekben az omega-3-zsírsavellátottság befolyásolásához az alfa-linolénsav-szupplementáció dózisának 1 g/nap fölé történő növelése tűnik szükségesnek.
Intraoperative sonography to exclude thoracic injury
Authors: Baranyai Zsolt , Jósa Valéria , Jakab Ferenc , Szabó Győző János
Published: August 2007

A szerzők 29 éves, késsel hasba szúrt nőbeteg esetét ismertetik. A megkezdett sokktalanítás és tájékozódó jellegű képalkotó vizsgálatok után a hasüregben kimutatott folyadék és hypovolaemiás keringésmegingás miatt azonnali laparotomiát, gyomorvarratot és a lép eltávolítását végezték. A hasüreg átvizsgálásakor észlelték a bal rekesz centrális sérülését. A műtétet indikáló mellüregi szervsérülések kizárására, az explorativ thoracotomia elkerülésére intraoperatív szonográfiát végeztek. A transzducert és az akusztikus gélt steril plasztikzsákba helyezve, a hasüreg felől tekintették át a rekesz feletti szerveket. Az eljárással egyértelműen ki lehetett zárni a rekesz közeli intrathoracalis sérülést.
Theoretical foundations of protein chips and their possible use in medical research and diagnostics
Authors: Spisák Sándor , Molnár Béla , Galamb Orsolya , Sipos Ferenc , Tulassay Zsolt
Published: August 2007

Az mRNS expressziós chippel való vizsgálati módszer elterjedésével egyre nagyobb igény merült fel az eredmények fehérjechipekkel történő validálására. A szerzők a különböző fehérjechip-módszerek előnyeit, hátrányait, alkalmazási lehetőségeit, eddigi eredményeit mutatják be. A fehérjék chip- vagy array-alapú analízisének speciális gátjai a molekulák bomlékonysága, a nem specifikus kötődés lehetősége és az amplifikációs módszerek hiánya. Ma üvegtárgylemez-alapú nyomtatott, SELDI MS-alapú, elektroforézis-alapú és szövettani mikroblokkmódszerek különböztethetők meg. Az üvegalapú chipek előnye az egyszerű alkalmazhatóságuk, a kisebb költségük. A SELDI-alapú fehérjechipek viszonylag nagy költséggel (tömegspektrométer), kis anyagmennyiségből (<1 µg) is tudnak profilkülönbségből adódó eredményt adni. Az elektroforézis-alapú eljárások még nem kiforrottak. A szöveti mikroblokkok az egyes antitestek szöveti és betegségspecificitásának meghatározását teszik lehetővé. A fehérjechipek eddigi alkalmazása a szérumban a betegségspecifikus mintázatok felderítésére, a daganatspecifikus jelátviteli utak kutatására, az mRNS expressziós vizsgálatok eredményeinek ellenőrzésére, igazolására szolgáltak. A fehérjechipek érzékenységének, specificitásának növekedése, valamint a költségek és a szükséges minta mennyiségének csökkenése elvezethet rutindiagnosztikai felhasználásukhoz.
Erlotinib in second/third line treatment for non-small cell lung cancer (NSCLC)
Authors: Sárosi Veronika , Balikó Zoltán
Published: August 2007

A nem kissejtes tüdőrák előrehaladott eseteiben a citosztatikus kezelésnek a betegek túlélésére a csak tüneti kezeléssel szemben kicsiny, de értékelhető hatása van. A citosztatikus gyógyszereknek azonban számos mellékhatásuk van, többek között neuro-, nephrotoxicitás, myeloszuppresszió. A daganatbiológia jobb megismerése során a daganat növekedésében számos kulcsfontosságú molekuláris útvonal vált ismertté. A nem kissejtes tüdőrákban az epidermális növekedésifaktor-receptor tyrosin kinase gátlók (gefitinib, erlotinib) a kedvező hatás- és mellékhatásprofiljuk miatt már a klinikai gyakorlatba kerültek. A betegség jellemző paraméterei, melyek ezeknek a gyógyszereknek a várható hatását megjósolják, még nem teljesen tisztázottak, ezek meghatározása érdekében további klinikai vizsgálatokra van szükség. Az Expanded Access Program keretében lehetővé vált előrehaladott (III/B és IV. stádium) nem kissejtes tüdőrákos betegeknél az erlotinib adása. A szerzők betegeik közül két olyan esetet mutatnak be, melyek jól szemléltetik a gyógyszer második, illetőleg harmadik vonalbeli alkalmazásának kedvező hatását. A szövettani minták részletesebb (epidermális növekedésifaktor-receptor expresszió mellett epidermális növekedésifaktor-receptor gén mutáció) vizsgálatának eredményei további adatokkal szolgálnak az erlotinib várható hatásossága tekintetében.
Adenomatoid tumor of adrenal gland. Case report and review of the literature
Authors: Füredi Gábor , Szilágyi Anna , Bencsik Zsuzsa , Altorjay Áron
Published: August 2007

A mellékvese adenomatoid tumora igen ritka, benignus, aszimptomatikus, általában véletlenül felfedezett elváltozás. A szerzők esetismertetésükben egy 32 éves férfi kórtörténetét mutatják be, akit láz és deréktáji fájdalmak miatt vizsgáltak. A kivizsgálás részét képező hasi UH-, majd az ezt követő hasi CT-vizsgálat igazolta a jobb mellékvese térfoglaló elváltozását. A betegnél adrenalectomiát végeztek, a szövettani vizsgálat a jobb mellékvese adenomatoid tumorát véleményezte. Az eset kapcsán áttekintik – az irodalmi adatok alapján – az adenomatoid tumorok előfordulási gyakoriságát, tünettanát, a diagnosztika-differenciáldiagnosztika buktatóit, a kezelési lehetőségeket és a prognózist.
Radio-Echo Sounding Over Polar Ice Masses
Authors: Bingham Robert G. , Siegert Martin J.
Published: August 2007

Radio-echo sounding (RES) constitutes the principal means by which glaciologists investigate the subsurface properties of the polar ice sheets and ice caps. Developed in the 1960s as a method for locating and mapping the subglacial interface beneath extensive regions of ice-covered terrain, thereby to constrain ice volume and morphology, it was quickly discovered that RES supplies numerous additional cryospheric parameters, including strong reflectors derived from subglacial lakes, and isochronous internal reflectors derived from burial of snow deposition events. Soon after its establishment, RES was integrated into long-range aircraft primarily to image the bed across Antarctica and Greenland (1960s∕1970s). More recent airborne campaigns (1980s∕1990s), while supplementing this coverage and extending to the ice caps of the High Arctic, have utilised only short-range aircraft, and were designed explicitly to support specific scientific studies, such as locating optimal sites for deep ice-coring, constraining the dimensions of subglacial lakes, or resolving internal layers for studies of ice sheet mass balance, form and flow. In parallel with these developments, ground-based (over-snow) RES equipment has also been used to investigate the englacial and subglacial conditions at a number of key locations across the polar ice sheets. This article discusses the many scientific advances which have resulted from these efforts, and offers recommendations for future developments in terms of (i) reanalysis of existing data and (ii) suggestions for future RES campaigns.
Short- and long-term glycaemic control and the state of the renin system in type 1 diabetes mellitus
Authors: Stevanovic Radomir D , Fisher Naomi DL , Lansang Cecilia M , Freeman Katherine D , Hollenberg Norman K
Published: August 2007

Renin system blockade in diabetes exerts a strong positive influence on complications, especially nephropathy. In hyperglycaemic diabetic subjects, however, blockade of the renin-angiotensin system with angiotensin-converting enzyme inhibitors results in a marked rise in plasma renin.We investigated whether glycaemic fluctuations measured in hours, or those measured in weeks by Haemoglobin A1C (HbA1C) , influenced the plasma renin response to captopril. Fifty-four type 1 diabetic subjects were studied in high-salt balance. After an all night fast and in the supine position, baseline serum glucose level was drawn. Iv. glucose and insulin were then administered to keep serum glucose between 100 and 150 mg/dL (target). When target was reached, captopril 25 mg pre os was administered and plasma renin activity (PRA) and finger stick glucose were drawn, then serially every 45 minutes for 225 minutes. Baseline glucose and baseline PRA were drawn hours apart. Peak PRA corresponded to the renin level at peak captopril effect, 90' after administration. Renin response (RR) = peak PRA — baseline PRA. Correlation of baseline glucose with baseline PRA was weak (r=0.3, p=0.02), but strong with peak PRA (r=0.65; p=0 P .002). Drop in glucose had a weak, negative correlation with baseline PRA (r=-0.3, p=0.03) but a much stronger one with peak PRA (r=-0.7, p<0.0001).After adjustment for baseline PRA and baseline glucose, mean RR correlated strongly with mean drop in glucose (r=-0.72; p=0.008). Conversely, HbA1C correlated with none of the measures of renin system activation (r=0.05;p=0.7).In type 1 diabetic subjects, short-term hyperglycaemia, but not long-term glycaemic control, enhanced the RR to captopril.
From One‐ to Three‐Dimensional Organic Semiconductors: In Search of the Organic Silicon?
Authors: Roncali J. , Leriche P. , Cravino A.
Published: August 2007

Organic semiconductors based on π‐conjugated systems are the focus of considerable interest in the emerging area of soft or flexible photonics and electronics. Whereas in recent years the performances of devices such as organic light‐emitting diodes (OLEDs), organic field‐effect transistors (OFETs), or solar cells have undergone considerable progress, a number of technical and fundamental problems related to the low dimensionality of organic semiconductors based on linear π‐conjugated systems remain unsatisfactorily resolved. This low dimensionality results in an anisotropy of the optical and charge‐transport properties, which in turn implies a control of the material organization/molecular orientation during or after device fabrication. Such a constraint evidently represents a problem when device fabrication by solution‐based processes, such as printing techniques, is envisioned. The aim of this short Review is to illustrate possible alternative strategies based on the development of organic semiconductors with higher dimensionality, capable to exhibit isotropic electronic properties.
Cover Picture: Conducting and Superhydrophobic Rambutan‐like Hollow Spheres of Polyaniline (Adv. Mater. 16/2007)
Authors: Zhu Y. , Hu D. , Wan M. X. , Jiang L. , Wei Y.
Published: August 2007

Conductive and superhydrophobic rambutan‐like hollow spheres of polyaniline are formed through self‐assembly by using perfluorooctane sulfonic acid as a dopant, soft template, and superhydrophobic agent at the same time, as reported by Meixiang Wan and Lei Jiang on p. 2092. The results show that the approach used is not only simple, but also that the hollow spheres have a large specific area and exhibit physical properties that are required for many applications in nanotechnology.
Prevalence of overweight and cardiovascular risk factors in rural and urban children from Central Asia: The Kazakhstan health and nutrition examination survey
Authors: Facchini Fiorenzo , Fiori Giovanni , Bedogni Giorgio , Galletti Livia , Belcastro Maria Giovanna , Ismagulov Orazak , Ismagulova Ainagul , Sharmanov Turegeldy , Tsoy Igor , Rizzoli Sara , Goldoni Matteo
Published: August 2007

Kazakhstan is undergoing a rapid modernization process, which carries the risk of an epidemic of obesity and cardiovascular disease. We enrolled a sample of about 50 children for every combination of gender, environment (urban vs. rural), ethnic group (Kazakh vs. Russian), and age group from 7 to 18 years, for a total of 4,808 children. Anthropometry and blood pressure were measured on all children while fasting blood cholesterol and glucose were measured only in 2,616 children aged ≥12 years. The prevalence of overweight and risk of overweight ranged from 2.8 (rural male Kazakhs) to 9.1% (urban male Russians). The prevalence of prehypertension and hypertension ranged from 8.3 (urban females) to 15.9% (rural females); that of hypercholesterolemia from 11.5 (male rural Russians) to 26.5% (female rural Kazakhs); and the overall prevalence of impaired fasting glucose was 0.1%. We conclude that overweight and cardiovascular risk factors are less prevalent in children living in Kazakhstan than in those living in Western countries. However, these figures are not negligible and suggest that preventive measures are needed to contain the epidemic of overweight and cardiovascular disease that will most likely accompany the modernization of Kazakhstan in the next years. Am. J. Hum. Biol., 2007. © 2007 Wiley‐Liss, Inc.
Early life influences on adult leg and trunk length in the 1958 British birth cohort
Authors: Li Leah , Dangour Alan D. , Power Chris
Published: August 2007

Objectives: Short leg length has been associated with increased disease risk. We investigated (1) whether taller childhood stature predicts longer adult leg than trunk length; (2) the effects of early life factors on adult leg/trunk length. Methods: We used data from the 1958 British birth cohort on height in childhood and at 45 years, leg and trunk length at 45 years and early life factors (n≈ 5,900). Results: For a SD increase in height at 7 years, adult leg length increased more than trunk length (2.5–2.8 cm vs. 1.9 cm). Parental height had a stronger association with adult than childhood height, and leg than trunk length. Prenatal factors were associated with leg (maternal smoking) and trunk length (birth order); birth weight had a similar effect on leg and trunk lengths. Large family size, overcrowding, and social housing were more strongly associated with leg than trunk length: deficits in adult height (0.4–0.8 cm) were mostly due to shorter legs. Conclusions: Socio‐economic adversity in childhood is associated with delayed early growth, shorter adult leg length, and stature. Leg length is the height component most sensitive to early environment. Studies of early life and adult disease could usefully assess adult leg length in addition to height. Am. J. Hum. Biol., 2007. © 2007 Wiley‐Liss, Inc.
Two unique patients with novel microdeletions in 4p16.3 that exclude the WHS critical regions: Implications for critical region designation
Authors: South Sarah T. , Bleyl Steven B. , Carey John C.
Published: August 2007

Wolf‐Hirschhorn syndrome (WHS) is characterized by growth delay, developmental delay, hypotonia, seizures, feeding difficulties, and characteristic facial features. Deletion of either of two critical regions (WHSCR and WHSCR‐2) within chromosome band 4p16.3 has been proposed as necessary for the minimal clinical manifestations of WHS and controversy remains regarding their designation. We describe two patients with novel terminal microdeletions in 4p16.3 who lack the characteristic facial features but do show some of the more nonspecific manifestations of WHS. The first patient had a ring chromosome 4 with an intact 4q subtelomere and a terminal 4p microdeletion of approximately 1.27–1.46 Mb. This deletion was distal to both proposed critical regions. The second patient had a normal karyotype with a terminal 4p microdeletion of approximately 1.78 Mb. This deletion was distal to WHSCR and the breakpoint was near or within the known distal boundary for WHSCR‐2. Both patients showed significant postnatal growth delay, mild developmental delays and feeding difficulties. Their facial features were not typical for WHS. The phenotype of the first patient may have been influenced by the presence of a ring chromosome. Seizures were absent in the first patient whereas the second patient had a complex seizure disorder. Characterization of these patients supports the hypothesis that a gene in WHSCR‐2,LETM1, plays a direct role in seizure development, and demonstrates that components of the WHS phenotype can be seen with deletions distal to the known boundaries of the two proposed critical regions. These patients also emphasize the difficulty of mapping clinical manifestations common to many aneusomy syndromes. © 2007 Wiley‐Liss, Inc.
Health‐related quality of life in multiple sclerosis: effects of natalizumab
Authors: Rudick Richard A. , Miller Deborah , Hass Steve , Hutchinson Michael , Calabresi Peter A. , Confavreux Christian , Galetta Steven L. , Giovannoni Gavin , Havrdova Eva , Kappos Ludwig , Lublin Fred D. , Miller David H. , O'Connor Paul W. , Phillips J. Theodore , Polman Chris H. , Radue Ernst‐Wilhelm , Stuart William H. , Wajgt Andrzej , Weinstock‐Guttman Bianca , Wynn Daniel R. , Lynn Frances , Panzara Michael A.
Published: August 2007

Objective

To report the relationship between disease activity and health‐related quality of life (HRQoL) in relapsing multiple sclerosis, and the impact of natalizumab.

Methods

HRQoL data were available from 2,113 multiple sclerosis patients in natalizumab clinical studies. In the Natalizumab Safety and Efficacy in Relapsing Remitting Multiple Sclerosis (AFFIRM) study, patients received natalizumab 300mg (n = 627) or placebo (n = 315); in the Safety and Efficacy of Natalizumab in Combination with Interferon Beta‐1a in Patients with Relapsing Remitting Multiple Sclerosis (SENTINEL) study, patients received interferon beta‐1a (IFN‐β‐1a) plus natalizumab 300mg (n = 589), or IFN‐β‐1a plus placebo (n = 582). The Short Form‐36 (SF‐36) and a subject global assessment visual analog scale were administered at baseline and weeks 24, 52, and 104. Prespecified analyses included changes from baseline to week 104 in SF‐36 and visual analog scale scores. Odds ratios for clinically meaningful improvement or worsening on the SF‐36 Physical Component Summary (PCS) and Mental Component Summary were calculated.

Results

Mean baseline SF‐36 scores were significantly less than the general US population and correlated with Expanded Disability Status Scale scores, sustained disability progression, relapse number, and increased volume of brain magnetic resonance imaging lesions. Natalizumab significantly improved SF‐36 PCS and Mental Component Summary scores at week 104 in AFFIRM. PCS changes were significantly improved by week 24 and at all subsequent time points. Natalizumab‐treated patients in both studies were more likely to experience clinically important improvement and less likely to experience clinically important deterioration on the SF‐36 PCS. The visual analog scale also showed significantly improved HRQoL with natalizumab.

Interpretation

HRQoL was impaired in relapsing multiple sclerosis patients, correlated with severity of disease as measured by neurological ratings or magnetic resonance imaging, and improved significantly with natalizumab. Ann Neurol 2007
Extended spectrum of idiopathic generalized epilepsies associated with CACNA1H functional variants
Authors: Heron Sarah E. , Khosravani Houman , Varela Diego , Bladen Chris , Williams Tristiana C. , Newman Michelle R. , Scheffer Ingrid E. , Berkovic Samuel F. , Mulley John C. , Zamponi Gerald W.
Published: August 2007

Objective

The relationship between genetic variation in the T‐type calcium channel gene CACNA1H and childhood absence epilepsy is well established. The purpose of this study was to investigate the range of epilepsy syndromes for which CACNA1H variants may contribute to the genetic susceptibility architecture and determine the electrophysiological effects of these variants in relation to proposed mechanisms underlying seizures.

Methods

Exons 3 to 35 of CACNA1H were screened for variants in 240 epilepsy patients (167 unrelated) and 95 control subjects by single‐stranded conformation analysis followed by direct sequencing. Cascade testing of families was done by sequencing or single‐stranded conformation analysis. Selected variants were introduced into the CACNA1H protein by site‐directed mutagenesis. Constructs were transiently transfected into human embryo kidney cells, and electrophysiological data were acquired.

Results

More than 100 variants were detected, including 19 novel variants leading to amino acid changes in subjects with phenotypes including childhood absence, juvenile absence, juvenile myoclonic and myoclonic astatic epilepsies, as well as febrile seizures and temporal lobe epilepsy. Electrophysiological analysis of 11 variants showed that 9 altered channel properties, generally in ways that would be predicted to increase calcium current.

Interpretation

Variants in CACNA1H that alter channel properties are present in patients with various generalized epilepsy syndromes. We propose that these variants contribute to an individual's susceptibility to epilepsy but are not sufficient to cause epilepsy on their own. The genetic architecture is dominated by rare functional variants; therefore, CACNA1H would not be easily identified as a susceptibility gene by a genome‐wide case–control study seeking a statistical association. Ann Neurol 2007
Early growth in brain volume is preserved in the majority of preterm infants
Authors: Boardman James P. , Counsell Serena J. , Rueckert Daniel , Hajnal Jo V. , Bhatia Kanwal K. , Srinivasan Latha , Kapellou Olga , Aljabar Paul , Dyet Leigh E. , Rutherford Mary A. , Allsop Joanna M. , Edwards A. David
Published: August 2007

Objective

Preterm infants have reduced cerebral tissue volumes in adolescence. This study addresses the question: Is reduced global brain growth in the neonatal period inevitable after premature birth, or is it associated with specific medical risk factors?

Methods

Eighty‐nine preterm infants at term equivalent age without focal parenchymal brain lesions were studied with 20 full‐term control infants. Using a deformation‐based morphometric approach, we transformed images to a reference anatomic space, and we used the transformations to calculate whole‐brain volume and ventricular volume for each subject. Patterns of volume difference were correlated with clinical data.

Results

Cerebral volume is not reduced compared with term born control infants (p = 0.765). Supplemental oxygen requirement at 28 postnatal days is associated with lower cerebral tissue volume at term (p < 0.001), but there were no significant differences in cerebral volumes attributable to perinatal sepsis (p = 0.515) and quantitatively defined diffuse white matter injury (p = 0.183). As expected, the ventricular system is significantly larger in preterm infants at term equivalent age compared with term control infants (p < 0.001).

Interpretation

Cerebral volume is not reduced during intensive care for the majority of preterm infants, but prolonged supplemental oxygen dependence is a risk factor for early attenuation of global brain growth. The reduced cerebral tissue volume seen in adolescents born preterm does not appear to be an inevitable association of prematurity, but rather caused by either specific disease during intensive care or factors operating beyond the neonatal period. Ann Neurol 2007
Disruption of cerebral cortex MET signaling in autism spectrum disorder
Authors: Campbell Daniel B. , D'Oronzio Rosanna , Garbett Krassi , Ebert Philip J. , Mirnics Karoly , Levitt Pat , Persico Antonio M.
Published: August 2007

Objective

Multiple genes contribute to autism spectrum disorder (ASD) susceptibility. One particularly promising candidate is the MET gene, which encodes a receptor tyrosine kinase that mediates hepatocyte growth factor (HGF) signaling in brain circuit formation, immune function, and gastrointestinal repair. The MET promoter variant rs1858830 allele “C” is strongly associated with ASD and results in reduced gene transcription. Here we examined expression levels of MET and members of the MET signaling pathway in postmortem cerebral cortex from ASD cases and healthy control subjects.

Methods

Protein, total RNA, and DNA were extracted from postmortem temporal cortex gray matter samples (BA 41/42, 52, or 22) belonging to eight pairs of ASD cases and matched control subjects. MET protein expression was determined by Western blotting; messenger RNA expression of MET and other related transcripts was assayed by microarray and quantitative reverse transcriptase polymerase chain reaction.

Results

MET protein levels were significantly decreased in ASD cases compared with control subjects. This was accompanied in ASD brains by increased messenger RNA expression for proteins involved in regulating MET signaling activity. Analyses of coexpression of MET and HGF demonstrated a positive correlation in control subjects that was disrupted in ASD cases.

Interpretation

Altered expression of MET and related molecules suggests dysregulation of signaling that may contribute to altered circuit formation and function in ASD. The complement of genes that encode proteins involved in MET activation appears to undergo long‐term compensatory changes in expression that may be a hallmark contribution to the pathophysiology of ASD. Ann Neurol 2007
COL4A1 mutation in Axenfeld–Rieger anomaly with leukoencephalopathy and stroke
Authors: Sibon Igor , Coupry Isabelle , Menegon Patrice , Bouchet Jean‐Pierre , Gorry Philippe , Burgelin Ingrid , Calvas Patrick , Orignac Isabelle , Dousset Vincent , Lacombe Didier , Orgogozo Jean‐Marc , Arveiler Benoît , Goizet Cyril
Published: August 2007

Objective

Several hereditary ischemic small‐vessel diseases of the brain have been reported during the last decade. Some of them have ophthalmological, mainly retinal, manifestations. Herein, we report on a family affected by vascular leukoencephalopathy and variable abnormalities of the anterior chamber of the eye.

Methods

After the occurrence of a small, deep infarct associated with white matter lesions in a patient with a medical history of congenital cataract and amblyopia, we conducted clinical and neuroradiological investigations in 10 of her relatives.

Results

Diffuse leukoencephalopathy associated with ocular malformations of the Axenfeld–Rieger type was observed in five individuals. Familial genetic analyses led to the identification of a novel missense mutation in the COL4A1 gene, p.G720D, which cosegregates with the disease.

Interpretation

Our data corroborate previous observations demonstrating the role of COL4A1 in cerebral microangiopathy and expand the phenotypic spectrum associated with mutations in this gene. We delineate a novel association between the Axenfeld–Rieger anomaly and leukoencephalopathy and stroke. Ann Neurol 2007
Mutations in the cyclic adenosine monophosphate response element of the tyrosine hydroxylase gene
Authors: Verbeek Marcel M. , Steenbergen‐Spanjers Gerry C. H. , Willemsen Michèl A. A. P. , Hol Frans A. , Smeitink Jan , Seeger Jürgen , Grattan‐Smith Padraic , Ryan Monique M. , Hoffmann Georg F. , Donati Maria A. , Blau Nenad , Wevers Ronald A.
Published: August 2007

Tyrosine hydroxylase (TH) deficiency (OMIM 191290) is one cause of early‐onset dopa‐responsive dystonia. We describe seven cases from five unrelated families with dopa‐responsive dystonia and low homovanillic acid in cerebrospinal fluid who were suspected to suffer from TH deficiency. Analysis of part of the TH promotor showed five homozygous and two heterozygous mutations in the highly conserved cyclic adenosine monophosphate response element. Our data suggest that, if no mutations are found in the coding regions of the gene in patients strongly suspected of TH deficiency, the search for pathogenic mutations should be extended to regulatory promotor elements. Ann Neurol 2007
Saccadic palsy after cardiac surgery: characteristics and pathogenesis
Authors: Solomon David , Ramat Stefano , Tomsak Robert L. , Reich Stephen G. , Shin Robert K. , Zee David S. , Leigh R. John
Published: August 2007

Objective

To characterize the syndrome of saccadic palsy that may follow cardiac surgery, and to interpret the findings using current concepts of the neurobiology of fast eye movements.

Methods

Using the magnetic search coil technique, we measured eye, eyelid, and head movements of 10 patients who developed selective palsy of saccades after cardiac surgery.

Results

Patients showed varying degrees of slowing and hypometria of saccades in the vertical plane or both horizontal and vertical planes, with complete loss of all saccades in one patient. Quick phases of nystagmus were also affected, but smooth pursuit, vergence, and the vestibuloocular reflex were usually spared. The smallest saccades were less slowed than larger saccades. Affected patients were visually disabled by loss of ability to voluntarily shift their direction of gaze. Blinks and head thrusts modestly improved the range and speed of voluntary movement. The syndrome usually followed aortic valve replacement. Common accompanying features included dysarthria, labile emotions, and unsteady gait. The saccadic palsy either improved during the early part of the course or remained static.

Interpretation

Selective loss of all forms of saccades, with sparing of other eye movements, indicates malfunction of the brainstem machinery that generates saccades. A current model of brainstem circuits could account for both hypometria and slowing. This syndrome and the visual disability it causes often go unrecognized unless saccades are systematically tested at the bedside. Ann Neurol 2007
Influence of ZnO nanoparticles on the cure characteristics and mechanical properties of carboxylated nitrile rubber
Authors: Sahoo Suchismita , Bhowmick Anil K.
Published: August 2007

Zinc oxide (ZnO) nanoparticles assembled in one dimension to give rod‐shaped morphology were synthesized. The effect of these ZnO nanoparticles (average particle size ∼ 50 nm) as the curing agent for carboxylated nitrile rubber was studied with special attention to cure characteristics, mechanical properties, dynamic mechanical properties, and swelling. These results were compared with those of the conventional rubber grade ZnO. The study confirmed that the ZnO nanoparticles gave a better state of cure and higher maximum torque with a marginal decrease in optimum cure time and scorch time. The mechanical properties also showed an improvement. There was an increase in tensile strength by ∼ 120%, elongation at break by ∼ 20%, and modulus at 300% elongation by ∼ 30% for the vulcanizate cured with ZnO nanoparticles, as compared with the one containing rubber grade ZnO. Dynamic mechanical analysis revealed that the vulcanizates exhibited two transitions—one occurring at lower temperature due to the Tg of the polymer, while the second at higher temperature corresponding to the hard phase arising due to the ionic structures. The second transition showed a peak broadening because of an increase in the points of interaction of ZnO nanoparticles with the matrix. The tan δ peak showed a shift towards higher Tg in the case of ZnO nanoparticle‐cured vulcanizate, indicating higher crosslinking density. This was further confirmed by volume fraction of rubber in the swollen gel and infrared spectroscopic studies. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007
Mechanism of filler action in reducing the wear of PTFE polymer by differential scanning calorimetry
Authors: Lai Shi‐Quan , Yue Li , Li Tong‐Sheng
Published: August 2007

Two types of representative nanometer materials, i.e., fibroid nanometer attapulgite and approximate spherical ultrafine diamond, were selected as fillers of polytetrafluoroethylene (PTFE) to study the mechanism of the wear‐reducing actions of the fillers in PTFE composites. The friction and wear tests were performed on a block‐on‐ring wear tester under dry sliding conditions. Differential scanning calorimetry (DSC) was used to investigate material microstructure and to examine modes of failure. No significant change in coefficient of friction was found, but the wear rate of PTFE composites was orders of magnitude less than that of pure PTFE. DSC analysis revealed that nanometer attapulgite and ultrafine diamond played a heterogeneous nucleation role in PTFE matrix and consequently resulted in increasing the crystallinity of PTFE composites. Moreover, the PTFE composite with higher heat absorption capacity and crystallinity exhibited improved wear resistance. A propositional “sea‐frusta” frictional model explained the wear mechanism of filler action in reducing the wear of PTFE polymer, i.e., fillers in the PTFE matrix effectively reduced the size of frictional broken units for PTFE composites and restrained the flowability of the units, as well as supporting the applied load. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007
Dual‐phase polymer electrolytes based on blending poly(MMA‐g‐NBR) and PMMA
Authors: Li Weili , Yang Mujie , Yuan Mingyong , Tang Zheng , Zhang J. Q.
Published: August 2007

A new type dual‐phase polymer electrolyte (DPE) based on poly(MMA‐g‐NBR) and poly(methyl methacrylate) (PMMA) was prepared by film‐casting method. In the DPE, PMMA phase is selectively impregnated with the lithium salt solution, forming an ion‐conducting network, while poly(MMA‐g‐NBR) phase maintains mechanical strength in the system. With grafting effect, the compatibility of two phases (PMMA/poly(MMA‐g‐NBR)) can be enhanced obviously. The chemical and physical characteristics, morphology and ionic conductivity behavior of DPEs, and their interfacial stability between lithium metal electrode were characterized by using of FTIR, 1HMNR, DSC, SEM, optical microscopic images, AC impedance, and linear sweep voltammetry. A new type DPE with good mechanics and high electrochemistry properties is obtained at an optimum proportion of two blending polymers. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007
Carboxyl‐terminated butadiene–acrylonitrile rubber modified cyanate ester resin
Authors: Shi Honghao , Fang Zhengping , Gu Aijuan , Tong Lifang , Xu Zhongbin
Published: August 2007

The carboxyl‐terminated butadiene‐a‐acrylonitrile rubber (CTBN) has been proved to be the most effective toughener for cyanate ester (CE) resin. This work mainly focuses on the different modification effects caused by the addition of CTBN with different acrylonitrile content. The phase separation, morphology of fracture surface, and physical properties of the blends are studied by scanning electron microscopy (SEM), transmission electron microscopy (TEM), dynamic mechanic analysis (DMA), and thermogravimetric analysis (TGA). It is testified that the compatibility and toughness between CE and CTBN had a positive correlation with the acrylonitrile content of CTBN. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007
Preparation and characterization of high‐density polyethylene/expanded graphite conducting masterbatch
Authors: Li Yuchao , Wu Dajun , Chen Guohua
Published: August 2007

High level of expanded graphite (EG) was melt‐blended with high‐density polyethylene to prepare electrical conducting masterbatch. Some factors such as processing temperature, EG contents, treating time were discussed for the effect on electrical and mechanical properties of composites. Results showed that EG tends to reunite while the content of EG is higher than 60% because of the large aspect ratio and surface area of EG nanosheets. In addition, increasing processing temperature and mixing time appropriately could enhance the dispersion of EG, leading to improvement in electrical and mechanical properties. Scanning electron microscopy (SEM) measurements were used as an assistant analysis to study the microstructure of composites. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007
Curing of silylated polyurethane with BF3‐monoethylamine complex as moisture‐curable adhesives and their properties
Authors: Nomura Yukihiro , Sato Shinichi , Mori Hideharu , Endo Takeshi
Published: August 2007

Boron trifluoride monoethylamine complex (BF3‐MEA) was evaluated as one of the curing catalyst, for the moisture‐curable adhesive based on the polyurethane end‐capped with trimethoxysilane (silylated polyurethane). BF3‐MEA worked as the catalyst of condensation reaction of trimethoxysilyl groups in silylated polyurethane to give the corresponding networked polymer. The curing speed, the bond strength, and the heat resistance of the crosslinked adhesives with BF3‐MEA were compared with dibutyltin dimethoxide. It was found that BF3‐MEA was a good curing catalyst, which showed the same performance in the bond strength and remarkable advantages in the curing speed and the heat resistance, compared with organotin compound. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007