bluuebunny/crossref_metadata_2025_split huggingface dataset:
Methane steam reforming at microscales: Operation strategies for variable power output at millisecond contact times
Authors: Stefanidis Georgios D. , Vlachos Dionisios G. , Kaisare Niket S. , Maestri Matteo 
Published: November 2008

AbstractThe potential of methane steam reforming at microscale is theoretically explored. To this end, a multifunctional catalytic plate microreactor, comprising of a propane combustion channel and a methane steam reforming channel, separated by a solid wall, is simulated with a pseudo 2‐D (two‐dimensional) reactor model. Newly developed lumped kinetic rate expressions for both processes, obtained from a posteriori reduction of detailed microkinetic models, are used. It is shown that the steam reforming at millisecond contact times is feasible at microscale, and in agreement with a recent experimental report. Furthermore, the attainable operating regions delimited from the materials stability limit, the breakthrough limit, and the maximum power output limit are mapped out. A simple operation strategy is presented for obtaining variable power output along the breakthrough line (a nearly iso‐flow rate ratio line), while ensuring good overlap of reaction zones, and provide guidelines for reactor sizing. Finally, it is shown that the choice of the wall material depends on the targeted operating regime. Low‐conductivity materials increase the methane conversion and power output at the expense of higher wall temperatures and steeper temperature gradients along the wall. For operation close to the breakthrough limit, intermediate conductivity materials, such as stainless steel, offer a good compromise between methane conversion and wall temperature. Even without recuperative heat exchange, the thermal efficiency of the multifunctional device and the reformer approaches ∼65% and ∼85%, respectively. © 2008 American Institute of Chemical Engineers AIChE J, 2009
Somatic TP53 mutation mosaicism in a patient with Li–Fraumeni syndrome
Authors: Prochazkova Kamila , Pavlikova Kristyna , Minarik Marek , Sumerauer David , Kodet Roman , Sedlacek Zdenek 
Published: November 2008

AbstractWe present a girl who developed adrenocortical adenoma at the age of 1 year and osteosarcoma at the age of 5 years. There was no history of cancer in her parents and their relatives. However, both tumors were typical for the Li–Fraumeni syndrome (LFS), and the patient met criteria for germline TP53 mutation testing. A mutation in codon 282 (Arg282Trp) was identified in her blood lymphocyte genomic DNA. The substitution was found in neither of her parents, which indicated a possibility of a de novo mutation. Unexpectedly, sequencing of the DNA of the patient repeatedly showed allelic imbalance in favor of the normal allele. This observation prompted us to investigate the putative somatic mosaicism in the patient consisting of normal cells and cells heterozygous for the mutation. The imbalance was also examined in two other non‐invasively sampled tissues, buccal cells, and cells from the urine sediment, and sequencing was confirmed with two other independent methods. While the findings in blood and the urine sediment were similar, in buccal cells both alleles were present in equal amounts. The allele ratio in lymphocytes was consistent with a mosaic where about 2/3 of cells carried two normal alleles and only 1/3 was heterozygous for the mutation. Despite the mosaicism the girl developed two early childhood tumors of mesodermal origin, and her phenotype was thus not milder than that of other germline TP53 mutation carriers. To our knowledge this is the first description of somatic mosaicism for a de novo TP53 mutation in LFS. © 2008 Wiley‐Liss, Inc.
Unique mosaic X/Y translocation/insertion in infant 45,X male
Authors: Chernykh V.B. , Vyatkina S.V. , Antonenko V.G. , Shilova N.V. , Zolotukhina T.V. , Kurilo L.F. , Chukhrova A.L. , Polyakov A.V. 
Published: November 2008

AbstractWe report on a 45,X male with hydrocephaly, lobar holoprosencephaly and ichthyosis. In situ hybridization and molecular analysis have demonstrated the presence of a mosaic SRY‐bearing derivative X chromosome that included Yp and heterochromatic Yq fragments. © 2008 Wiley‐Liss, Inc.
Developmental delay, dysmorphic features, neonatal spontaneous fractures, wrinkled skin, and hepatic failure: A new metabolic syndrome?
Authors: Mégarbané André , Samaras Leila , Chédid Rima , Chouery Eliane , Chrétien Dominique , Caillaud Catherine , Abou‐Ghoch Joelle , Jalkh Nadine 
Published: November 2008

AbstractWe report on a consanguineous Lebanese family where two sibs had an axial hypotonia, developmental delay, hirsutism, large fontanels with delayed closure, and dysmorphic facial features that consist of frontal bossing, prominent eyes, slightly down‐slanting palpebral fissures, hypertelorism, telecanthus, long eyelashes, gum hypertrophy, and pointed chin. In addition, they had short neck, abnormal thoracic configuration, wrinkled skin on the hands and abdomen, hepato‐splenomegaly and neonatal spontaneous fractures. Their overall health and hepatic function deteriorated every time they had fever. The eldest boy died at the age of 18 months secondary to a hepatic failure. Laboratory exams did not reveal any anomaly except for the hepatic function. Differential diagnoses are discussed and the possibility that we might be reporting on a new metabolic syndrome is raised. © 2008 Wiley‐Liss, Inc.
A novel 2.43 Mb deletion of 7q11.22–q11.23
Authors: Blyth Moira , Beal Sarah , Huang Shuwen , Crolla John , Foulds Nicola 
Published: November 2008

AbstractWe present a patient with a novel heterozygous deletion of 7q11.22–q11.23. Standard cytogenetic analysis using the ELN cosmid 82C and the ELN/ LIMK1 cosmid 34B FISH probes suggested a diagnosis of Williams syndrome. Although he has supravalvular aortic stenosis and peripheral pulmonary artery stenosis, which are common in this condition, he does not have the clinical gestalt of Williams syndrome. 44k oligo array CGH analysis showed a 2.43 Mb deletion, encompassing the proximal 1.43 kb of the Williams syndrome critical region and extending approximately 1 Mb beyond it. The deletion of further genes outside the Williams syndrome critical region does not appear to be having a phenotypic effect at present. © 2008 Wiley‐Liss, Inc.
Predictive genetic testing for cardiovascular diseases: Impact on carrier children
Authors: Meulenkamp Tineke M. , Tibben Aad , Mollema Eline D. , van Langen Irene M. , Wiegman Albert , de Wert Guido M. , de Beaufort Inez D. , Wilde Arthur A.M. , Smets Ellen M.A. 
Published: November 2008

AbstractWe studied the experiences of children identified by family screening who were found to be a mutation carrier for a genetic cardiovascular disease (Long QT Syndrome (LQTS), Hypertrophic Cardiomyopathy (HCM), Familial Hypercholesterolemia (FH)). We addressed the (a) manner in which they perceive their carrier status, (b) impact on their daily lives, and (c) strategy used to cope with these consequences. Children (aged 8–18) who tested positive for LQTS (n = 11), HCM (n = 6) or FH (n = 16), and their parents participated in semi‐structured audiotaped interviews. Interview topics included illness perception, use of medication, lifestyle modifications, worries, and coping. Each interview was coded by two researchers. The qualitative analysis was guided by Leventhal's model of self‐regulation. The children were overall quite articulate about the disease they were tested for, including its mode of inheritance. They expressed positive future health perceptions, but feelings of controllability varied. Adherence and side‐effects were significant themes with regard to medication‐use. Refraining from activities and maintaining a non‐fat diet were themes concerning lifestyle modifications. Some children spontaneously reported worries about the possibility of dying and frustration about being different from peers. Children coped with these worries by expressing faith in the effectiveness of medication, trying to be similar to peers or, in contrast, emphasizing their “being different.” Children generally appeared effective in the way they coped with their carrier status and its implications. Nevertheless, dealing with the daily implications of their condition remains difficult in some situations, warranting continued availability of psychosocial support. © 2008 Wiley‐Liss, Inc.
The presence of bicuspid aortic valve does not predict ventricular septal defect type
Authors: Hor Kan N. , Border William L. , Cripe Linda H. , Benson D. Woodrow , Hinton Robert B. 
Published: November 2008

AbstractPrevious studies have identified an increased incidence of bicuspid aortic valve (BAV) in patients with ventricular septal defect (VSD). Because endocardial cushion remodeling contributes to both the formation of semilunar valves and ventricular septation, we hypothesized that examination of humans with BAV and VSD would identify a specific VSD type. We evaluated VSD type in pediatric patients diagnosed with BAV and VSD (n = 82) and compared findings to patients diagnosed with VSD and normal aortic valve morphology (n = 429). VSD type was described as conoventricular, muscular, inlet or conoseptal using a clinical taxonomy. Based on the contribution of the outflow tract endocardial cushions to the membranous ventricular septum, we expected patients with BAV to have conoventricular VSD. In both patient groups, conoventricular VSD was most common; however, the prevalence was not significantly different when BAV patients were compared to those with normal aortic valve morphology (67% vs. 57%, P = 0.11). The primary finding of this study is that despite a developmental link between semilunar valve formation and ventricular septation during cardiogenesis, there is no clear association between BAV and VSD type. This may be due to phenotypic and genetic heterogeneity of BAV and VSD, other modifying factors as manifested by differences in associated CVM, as well as limitations of the clinical taxonomy of VSD. © 2008 Wiley‐Liss, Inc.
Band‐like intracranial calcification with simplified gyration and polymicrogyria: A distinct “pseudo‐TORCH” phenotype
Authors: Briggs T.A. , Wolf N.I. , D'Arrigo S. , Ebinger F. , Harting I. , Dobyns W.B. , Livingston J.H. , Rice G.I. , Crooks D. , Rowland‐Hill C.A. , Squier W. , Stoodley N. , Pilz D.T. , Crow Y.J. 
Published: November 2008

AbstractThe combination of intracranial calcification and polymicrogyria is usually seen in the context of intrauterine infection, most frequently due to cytomegalovirus. Rare familial occurrences have been reported. We describe five patients—two male–female sibling pairs, one pair born to consanguineous parents, and an unrelated female—with a distinct pattern of band‐like intracranial calcification associated with simplified gyration and polymicrogyria. Clinical features include severe post‐natal microcephaly, seizures and profound developmental arrest. Testing for infectious agents was negative. We consider that these children have the same recognizable “pseudo‐TORCH” phenotype inherited as an autosomal recessive trait. © 2008 Wiley‐Liss, Inc.
Clinical and molecular characterization of a patient with Langer–Giedion syndrome and mosaic del(8)(q22.3q24.13)
Authors: Shanske Alan L. , Patel Ankita , Saukam Sou , Levy Brynn , Lüdecke Hermann‐Josef 
Published: November 2008

AbstractThe tricho‐rhino‐phalangeal syndrome type II (TRPS II) is characterized by sparse scalp hair, a long nose with a bulbous tip, a long flat philtrum, cone‐shaped epiphyses of the phalanges, retarded bone age in infancy and multiple cartilaginous exostoses. All patients have a hemizygous deletion on chromosome 8q23.3‐24.11 which spans at least the 2.8 Mb‐region from TRPS1 through EXT1. Only patients with deletions that extend beyond this interval tend to have mental retardation. Here we describe a 14.5‐year‐old girl with mental retardation and TRPS II. Her facial features are only mild, but she has the typical skeletal features including cone‐shaped epiphyses at the phalanges, retarded bone age, multiple exostoses and short stature. She is the first patient with TRPS II and a molecularly proven mosaic interstitial deletion in 8q22.3‐q24.13. The deletion is one of the largest ever found in TRPS II, and spans 19.79 Mb and 50 genes or loci including TRPS1 and EXT1. The degree of mosaicism is 7% in lymphocytes from peripheral blood and 97% in skin fibroblasts. © 2008 Wiley‐Liss, Inc.
Sexual behavior across ovarian cycles in wild black howler monkeys (Alouatta pigra): male mate guarding and female mate choice
Authors: Van Belle Sarie , Estrada Alejandro , Ziegler Toni E. , Strier Karen B. 
Published: November 2008

AbstractWe studied two multimale–multifemale groups of black howler monkeys (Alouatta pigra) during a 14‐month study (June 2006–July 2007) in Palenque National Park, Mexico to evaluate the ways in which their sexual behavior changes across ovarian cycles. We analyzed 231 fecal samples, collected every 2.2±1.4 days from five females. For four females, estradiol and progesterone profiles revealed an average (±SE) cycle length of 18.3±1.4 days. Copulations occurred significantly more frequently during the periovulatory period (POP), defined as the estimated day of ovulation ±3 days (N=18). This was largely the result of cycling females soliciting sexual interactions during their POPs. Females directed their solicitations significantly more often toward “central” males of their group, who had close spatial associations with females at other times, compared with “noncentral” males, who did not associate closely with females. Central males rarely solicited sexual interactions, but instead monitored the females' reproductive status by sniffing their genitals, and maintained significantly closer proximity to females during their POPs, suggesting male mate guarding when conceptions are most likely to occur. Our findings indicate that the reproductive strategies of black howler central males and females coincide, highly skewing mating opportunities toward central males. Black howler females, however, occasionally choose to copulate with noncentral resident males or extra‐group males during their POPs, undermining the ability of central males to monopolize all reproductive opportunities. Am. J. Primatol. 71:153–164, 2009. © 2008 Wiley‐Liss, Inc.
Decision anomalies, experimenter assumptions, and participants' comprehension: Revaluating the uncertainty effect
Authors: Keren Gideon , Willemsen Martijn C. 
Published: November 2008

AbstractIdentifying discrepancies between normative prescriptions and actual behavior constitutes an important facet of studying judgment and decision making. Notwithstanding, alleged normative violations should be submitted to the most stringent tests before their existence is recognized. The present paper centers on participants' understanding of instructions and comprehension of the experimental task. As an example, we examine the “uncertainty effect” which, supposedly, violates canonical requirements of the theory of rational choice. Following this effect, people often value a lottery less than its worst possible realization, due to the uncertainty associated with the lottery. We empirically demonstrate that Gneezy et al.'s instructions were ambiguous. We show that only participants who miscomprehended the instructions exhibited a response pattern consistent with the uncertainty effect. Removing the ambiguity results in the elimination of the effect. Broader implications for the judgment and decision making literature are briefly discussed. Copyright © 2008 John Wiley & Sons, Ltd.
Delivery of apoptotic signal to rolling cancer cells: A novel biomimetic technique using immobilized TRAIL and E‐selectin
Authors: Rana Kuldeepsinh , Liesveld Jane L. , King Michael R. 
Published: November 2008

AbstractThe survival rate for patients with metastases versus localized cancer is dramatically reduced, with most deaths being associated with the formation of secondary tumors. Circulating cancer cells interact with the endothelial lining of the vasculature via a series of adhesive interactions that facilitate tethering and firm adhesion of cancer cells in the initial steps of metastasis. TNF‐related apoptosis‐inducing ligand (TRAIL) holds promise as a tumor‐specific cancer therapeutic, by inducing a death signal by apoptosis via the caspase pathway. In this study, we exploit this phenomenon to deliver a receptor‐mediated apoptosis signal to leukemic cells adhesively rolling along a TRAIL and selectin‐bearing surface. Results show that cancer cells exhibit selectin‐mediated rolling in capillary flow chambers, and that the rolling velocities can be controlled by varying the selectin and selectin surface density and the applied shear stress. It was determined that a 1 h rolling exposure to a functionalized TRAIL and E‐selectin surface was sufficient to kill 30% of captured cells compared to static conditions in which 4 h exposure was necessary to kill 30% of the cells. Thus, we conclude that rolling delivery is more effective than static exposure to a TRAIL immobilized surface. We have also verified that there is no significant effect of TRAIL on hematopoietic stem cells and other normal blood cells. This represents the first demonstration of a novel biomimetic method to capture metastatic cells from circulation and deliver an apoptotic signal. Biotechnol. Bioeng. 2009;102: 1692–1702. © 2008 Wiley Periodicals, Inc.
Novel Diazirine‐Containing DNA Photoaffinity Probes for the Investigation of DNA‐Protein‐Interactions
Authors: Winnacker Malte , Breeger Sascha , Strasser Ralf , Carell Thomas 
Published: November 2008

AbstractAn investigation of the precise interactions between damaged DNA and DNA repair enzymes is required in order to understand the lesion recognition step, which is one of the most fundamental processes in DNA repair. Most recently, photoaffinity labeling approaches have enabled the analysis of even transient protein‐DNA interactions. Here we report the synthesis and evaluation of oligonucleotides that contain two photoaffinity “catcher moieties” next to incorporated DNA lesions. With these DNA constructs it is possible to analyze the interactions between DNA lesions and the appropriate repair enzymes. The probes labeled the repair protein efficiently enough to enable subsequent protein analysis by mass spectrometry.
Photocontrollable Peptide‐Based Switches Target the Anti‐Apoptotic Protein Bcl‐xL
Authors: Kneissl Sabine , Loveridge E. Joel , Williams Christopher , Crump Matthew P. , Allemann Rudolf K. 
Published: November 2008

AbstractPhotocontrol of Bcl‐xL binding affinity has been achieved by using short BH3 domain peptides for Bak72–87 and Bid91–111 alkylated with an azobenzene crosslinker through two cysteine residues with different sequence spacings. The power to control the conformation of the crosslinker and hence peptide structure was demonstrated by CD and UV/Vis spectroscopy. The binding affinity of the alkylated peptides with Bcl‐xL was determined in their dark‐adapted and irradiated states by fluorescence anisotropy measurements, and use of different cysteine spacings allowed either activation or deactivation of the binding activities of these peptide‐based switches by application of light pulses. Helix‐stabilized peptides exhibited high Bcl‐xL binding affinity with dissociation constants of 42±9, 21±1, and 55±4 nM for Bak${{{i+ 7\atop 72-87}}}$, Bak${{{i+ 11\atop 72-87}}}$, and Bid${{{i+ 4\atop 91-111}}}$, respectively (superscript numbers refer to the spacing between cysteine residues), and up to 20‐fold enhancements in affinity in relation to their helix‐destabilized forms. Bak${{{i+ 7\atop 72-87}}}$, Bak${{{i+ 11\atop 72-87}}}$, and Bid${{{i+ 4\atop 91-111}}}$ each displayed more than 200‐fold selectivity for binding to Bcl‐xL over Hdm2, which is targeted by the N‐terminal helix of the tumor suppressor p53. Structural studies by NMR spectroscopy demonstrated that the peptides bind to the same cleft in Bcl‐xL as the wild‐type peptide regardless of their structure. This work opens the possibility of using such photocontrollable peptide‐based switches to interfere reversibly and specifically with biomacromolecular interactions to study and modulate cellular function.
Formation of Diverse Mesophases Templated by a Diprotic Anionic Surfactant
Authors: Gao Chuanbo , Sakamoto Yasuhiro , Terasaki Osamu , Che Shunai 
Published: November 2008

AbstractThe synthesis system for mesophase formation, using the diprotic anionic surfactant N‐myristoyl‐L‐glutamic acid (C14GluA) as the structure‐directing agent (SDA) and N‐trimethoxylsilylpropyl‐N,N,N‐trimethylammonium chloride (TMAPS) as the co‐structure‐directing agent (CSDA), has been investigated and a full‐scaled synthesis‐field diagram is presented. In this system we have obtained mesophases including three‐dimensional (3D) micellar cubic Fm$\bar 3$m, Pm$\bar 3$n, Fd$\bar 3$m, micellar tetragonal P42/mnm, two‐dimensional (2D) hexagonal p6mm and bicontinuous cubic Pn$\bar 3$m, by varying the C14GluA/NaOH/TMAPS composition ratios. From the diagram it can be concluded that the mesophase formation is affected to a high degree by the organic/inorganic‐interface curvature and the mesocage–mesocage electrostatic interaction. Bicontinuous cubic and 2D‐hexagonal phases were found in the low organic/inorganic‐interface curvature zones, whereas micellar cubic and tetragonal mesophases were found in the high organic/inorganic‐interface curvature zones. Formation of cubic Fm$\bar 3$m and tetragonal P42/mnm was favoured in highly alkaline zones with strong mesocage–mesocage interactions, and formation of cubic Pm$\bar 3$n and Fd$\bar 3$m was favoured with moderate mesocage–mesocage interactions in the less alkaline zones of the diagram.
ChemInform Abstract: A Non‐Twinned Polymorph of CaTe2O5 from a Hydrothermally Grown Crystal.
Authors: Weil Matthias , Stoeger Berthold 
Published: November 2008

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract of an article which was published elsewhere, please select a “Full Text” option. The original article is trackable via the “References” option.
ChemInform Abstract: Tl4(PO2NH)4×H2O — A Commensurately Modulated Tetrametaphosphimate.
Authors: Sedlmaier Stefan J. , Oeckler Oliver , Schnick Wolfgang 
Published: November 2008

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract of an article which was published elsewhere, please select a “Full Text” option. The original article is trackable via the “References” option.
ChemInform Abstract: Isolation of Two‐Dimensional 2:1 Cation‐Ordered Perovskite Units by Anion Vacancy Ordering in Ba6Na2Nb2P2O17.
Authors: Kuang Xiaojun , Claridge John B. , Price Tim , Iddles David M. , Rosseinsky Matthew J. 
Published: November 2008

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract of an article which was published elsewhere, please select a “Full Text” option. The original article is trackable via the “References” option.
ChemInform Abstract: Polar Hexagonal Tungsten Bronze‐Type Oxides: KNbW2O9, RbNbW2O9, KTaW2O9.
Authors: Chang H. Y. , Sivakumar T. , Ok K. M. , Halasyamani P. Shiv 
Published: November 2008

AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract of an article which was published elsewhere, please select a “Full Text” option. The original article is trackable via the “References” option.