bluuebunny/crossref_metadata_2025_split huggingface dataset:
Behavioral features in young adults with FG syndrome (Opitz–Kaveggia syndrome)
Authors: Graham John M. , Clark Robin D. , Moeschler John B. , Rogers R. Curtis 
Published: October 2010

AbstractOpitz and Kaveggia [Opitz and Kaveggia (1974); Z Kinderheilkd 117:1–18] reported on a family of five affected males with distinctive facial appearance, mental retardation, macrocephaly, imperforate anus, and hypotonia. Risheg et al. [Risheg et al. (2007); Nature Genetics 39:451–453] identified an identical mutation (p.R961W) in MED12 in six families with Opitz–Kaveggia syndrome, including a surviving affected man from the original family reported in 1974. The previously described behavior phenotype of hyperactivity, affability, and excessive talkativeness is very frequent in young boys with FG syndrome, along with socially oriented, attention‐seeking behaviors. We present case studies of five adult males who were previously published with the clinical diagnosis of FG syndrome and then subsequently proven by Risheg et al. [Risheg et al. (2007); Nature Genetics 39:451–453] to have the recurrent p.R961W mutation. These individuals had episodic and longstanding behavior patterns, sometimes aggressive or self‐abusing, that occurred more frequently in puberty and early adulthood. We try to describe the triggers for these behaviors, indicate how these behaviors change with advancing age, and suggest specific recommendations and interventional strategies based on the clinical histories of affected adolescent males with FG syndrome [Graham et al., 2008; Clark et al., 2009]. Young men who exhibit these behaviors may benefit from a careful examination to detect medical problems, use of mood stabilizers if needed, and/or behavioral intervention. The transition to a community living situation can be challenging without careful planning and timely behavioral intervention. They remain impulsive and can have aggressive outbursts when making the transition to adult life, but these challenges can be managed, as demonstrated by these clinical histories. © 2010 Wiley‐Liss, Inc.
Disease exacerbation by etanercept in a mouse model of alphaviral arthritis and myositis
Authors: Zaid Ali , Rulli Nestor E. , Rolph Michael S. , Suhrbier Andreas , Mahalingam Suresh 
Published: October 2010

AbstractObjectiveMosquito‐borne alphaviruses such as chikungunya virus, o'nyong‐nyong virus, and Ross River virus (RRV) cause sporadic, sometimes large, outbreaks of rheumatic disease worldwide. This study was designed to test the effect of treating RRV‐induced arthritis using the anti–tumor necrosis factor (anti‐TNF) drug etanercept in a mouse model of rheumatic disease.MethodsMice were infected with RRV and treated with etanercept. Weight gain was measured, tissue viral titers were determined, and histologic changes in muscle and joint tissues were assessed.ResultsRRV‐infected mice treated with etanercept showed decreased weight gain, higher viral titers in muscle, joints, and blood, and more tissue damage and inflammatory cell recruitment than RRV‐infected mice without treatment.ConclusionAnti‐TNF therapy is unlikely to be useful in treating alphaviral arthritides. During alphaviral epidemics, careful monitoring of patients being treated with anti‐TNF agents may be warranted.
Treatment response, drug survival, and predictors thereof in 764 patients with psoriatic arthritis treated with anti–tumor necrosis factor α therapy: Results from the nationwide Danish DANBIO registry
Authors: Glintborg Bente , Østergaard Mikkel , Dreyer Lene , Krogh Niels Steen , Tarp Ulrik , Hansen Michael Sejer , Rifbjerg‐Madsen Signe , Lorenzen Tove , Hetland Merete Lund 
Published: October 2010

AbstractObjectiveTo investigate disease activity, treatment response, and drug survival, and predictors thereof, among Danish patients with psoriatic arthritis (PsA) receiving their first treatment series with a tumor necrosis factor α (TNFα) inhibitor.MethodsPatients with PsA were identified from a prospective nationwide rheumatologic database, the Danish biologics registry DANBIO, using data registered from 2000–2009. Information was obtained on the patients' clinical response to anti‐TNFα treatment (defined as achievement of the American College of Rheumatology 20% [ACR20], ACR50, and ACR70 improvement criteria or a European League Against Rheumatism [EULAR] good response at least once during the first 6 months of treatment) and duration and rate of drug adherence (referred to as drug survival), as well as predictors thereof.ResultsOf 764 patients with PsA, 320 received adalimumab, 260 infliximab, and 184 etanercept. Median drug survival was 2.9 years, and 1‐year and 2‐year drug survival rates were 70% and 57%, respectively. Clinical parameters that showed improvement over 6 months were the C‐reactive protein (CRP) level, Health Assessment Questionnaire score, and 28‐joint Disease Activity Score. Male sex, CRP level >10 mg/liter, concomitant methotrexate use, and low patient health visual analog scale score at baseline were associated with longer drug survival. Improvement was achieved by 59%, 45%, 24%, and 54% of patients according to the ACR20, ACR50, ACR70 response criteria and EULAR good response, respectively. A CRP level >10 mg/liter was predictive of the improvement responses (odds ratio [OR] 2.6 for ACR20, OR 3.0 for ACR50, OR 3.6 for ACR70, and OR 2.2 for EULAR good response).ConclusionIn these patients with PsA treated with their first TNFα inhibitor in clinical practice, high drug adherence and responder rates were observed. Moreover, increased levels of CRP at baseline were associated with both good treatment responses and continued treatment, which may be of clinical value in selecting the patients most likely to benefit from treatment with TNFα inhibitors.
Galectin 3 aggravates joint inflammation and destruction in antigen‐induced arthritis
Authors: Forsman Huamei , Islander Ulrika , Andréasson Emil , Andersson Annica , Önnheim Karin , Karlström Alexandra , Sävman Karin , Magnusson Mattias , Brown Kelly L. , Karlsson Anna 
Published: October 2010

AbstractObjectiveGalectin 3, an endogenous β‐galactoside–binding lectin, plays an important role in the modulation of immune responses. The finding that galectin 3 is present in the inflamed synovium in patients with rheumatoid arthritis suggests that the protein is associated with the pathogenesis of this disease. We undertook this study to investigate the influence of galectin 3 deficiency in a murine model of arthritis.MethodsWild‐type (WT) and galectin 3–deficient (galectin 3−/−) mice were subjected to antigen‐induced arthritis (AIA) through immunization with methylated bovine serum albumin. The concentration of serum cytokines (interleukin‐6 [IL‐6] and tumor necrosis factor α [TNFα]) and antigen‐specific antibodies was evaluated using a cytometric bead array platform and enzyme‐linked immunosorbent assay (ELISA). Cellular IL‐17 responses were examined by flow cytometry, ELISA, and enzyme‐linked immunospot assay.ResultsThe joint inflammation and bone erosion of AIA were markedly suppressed in galectin 3−/− mice as compared with WT mice. The reduced arthritis in galectin 3−/− mice was accompanied by decreased levels of antigen‐specific IgG and proinflammatory cytokines. The frequency of IL‐17–producing cells in the spleen was reduced in galectin 3−/− mice as compared with WT mice. Exogenously added recombinant galectin 3 could partially restore the reduced arthritis and cytokines in galectin 3−/− mice.ConclusionOur findings show that galectin 3 plays a pathogenic role in the development and progression of AIA and that the disease severity is accompanied by alterations of antigen‐specific IgG levels, systemic levels of TNFα and IL‐6, and frequency of IL‐17–producing T cells. To our knowledge, this is the first report of in vivo evidence that galectin 3 plays a crucial role in the development of arthritis.
Influence of growth temperature on the production of antibody Fab fragments in different microbes: A host comparative analysis
Authors: Dragosits Martin , Frascotti Gianni , Bernard‐Granger Lise , Vázquez Felícitas , Giuliani Maria , Baumann Kristin , Rodríguez‐Carmona Escarlata , Tokkanen Jaana , Parrilli Ermenegilda , Wiebe Marilyn G. , Kunert Renate , Maurer Michael , Gasser Brigitte , Sauer Michael , Branduardi Paola , Pakula Tiina , Saloheimo Markku , Penttilä Merja , Ferrer Pau , Luisa Tutino Maria , Villaverde Antonio , Porro Danilo , Mattanovich Diethard 
Published: October 2010

AbstractMicroorganisms encounter diverse stress conditions in their native habitats but also during fermentation processes, which have an impact on industrial process performance. These environmental stresses and the physiological reactions they trigger, including changes in the protein folding/secretion machinery, are highly interrelated. Thus, the investigation of environmental factors, which influence protein expression and secretion is still of great importance. Among all the possible stresses, temperature appears particularly important for bioreactor cultivation of recombinant hosts, as reductions of growth temperature have been reported to increase recombinant protein production in various host organisms. Therefore, the impact of temperature on the secretion of proteins with therapeutic interest, exemplified by a model antibody Fab fragment, was analyzed in five different microbial protein production hosts growing under steady‐state conditions in carbon‐limited chemostat cultivations. Secretory expression of the heterodimeric antibody Fab fragment was successful in all five microbial host systems, namely Saccharomyces cerevisiae, Pichia pastoris, Trichoderma reesei, Escherichia coli and Pseudoalteromonas haloplanktis. In this comparative analysis we show that a reduction of cultivation temperature during growth at constant growth rate had a positive effect on Fab 3H6 production in three of four analyzed microorganisms, indicating common physiological responses, which favor recombinant protein production in prokaryotic as well as eukaryotic microbes. © 2010 American Institute of Chemical Engineers Biotechnol. Prog., 2011
Genetic damage in multiple organs of acutely exercised rats
Authors: Pozzi Renan , Rosa Jose C , Eguchi Ricardo , Oller do Nascimento Claudia M , Oyama Lila M , Aguiar Odair , Chaves Marcelo D , Ribeiro Daniel A 
Published: October 2010

AbstractThe aim of this study was to investigate the effects of acute exercise on genomic damage in an animal model. Male adult Wistar rats were divided into the following groups: control and acute exercised (experimental). For this purpose, 15 animals were accustomed to running on a rodent treadmill for 15 min per day for 5 days (10–20 m min−1; 08 grade). After 4 days at rest, active animals ran on the treadmill (22 m min−1, 58 grade) till exhaustion. Cells from peripheral blood, liver, heart, and brain were collected after 0, 2, and 6 h after exercise. The results showed that acute exercise was able to induce genetic damage in peripheral blood cells after 2 and 6 h of exercise, whereas liver pointed out genetic damage for all periods evaluated. No genetic damage was induced either in brain or in heart cells. In conclusion, our results suggest that acute exercise could contribute to the genetic damage in peripheral blood and liver cells. It seems that liver is a sensitive organ to the genotoxic insult after acute exercise. Copyright © 2010 John Wiley & Sons, Ltd.
Mitochondrial Mode of Action of a Thymidine‐Based Cisplatin Analogue Breaks Resistance in Cancer Cells
Authors: Onambele Liliane A. , Koth Daniel , Czaplewska Justyna A. , Schubert Ulrich S. , Görls Helmar , Yano Shigenobu , Obata Makoto , Gottschaldt Michael , Prokop Aram 
Published: October 2010

AbstractAbstract: Cisplatin analogue complexes with platinum(II) and palladium(II) starting from 3′,5′‐diamino‐3′,5′‐dideoxy‐thymidines were synthesized, both with the D‐erythro‐ and D‐threo configurations. Complexes of the general formula [MCl2L] were obtained and characterized. NMR spectroscopic measurements and single crystal X‐ray structure analysis showed that the metal centers are coordinated to the ligands by the amino groups in 3′‐ and 5′‐positions and not through the thymine moiety. All ligands and complexes showed no significant in vitro activities except thymiplatin (cis‐dichloro(3′,5′‐diamino‐3′,5′‐dideoxy‐D‐threo‐thymidine)platinum(II)). Detailed in vitro studies on the apoptosis pathway in lymphoma (BJAB), leukemia (NALM‐6), and melanoma cells (Mel‐HO) as well as on transfected or resistant cell lines were carried out. Thymiplatin significantly induced an apoptotic response, which was found to be associated with the loss of mitochondrial membrane potential and with caspase activation. The activity was shown to be independent of Fas‐associated protein with death domain (FADD), but dependent on Bcl‐2 expression. As a consequence, for thymiplatin a mitochondrial mode of action could be assigned. Moreover, the compound showed activity in cells resistant to common drugs, such as daunorubicin and vincristin, and showed synergistic effects with doxorubicin, vincristin, cytarabin, and daunorubicin.
Probing the Influence of an Allylic Methyl Group in Zearalenone Analogues on Binding to Hsp90
Authors: Rink Christian , Sasse Florenz , Zubrienė Asta , Matulis Daumantas , Maier Martin E. 
Published: October 2010

AbstractBy the replacement of an acetate with propionate by means of organic synthesis, a range of zearalenone analogues were prepared that feature an allylic methyl group. For the synthesis of the aliphatic region of the analogues, we used an asymmetric alkylation to yield pentenol derivatives 16 and ent‐16. By means of hydroboration the corresponding aldehydes were secured. These were coupled with 2‐pentynol derivate 23 by means of a Carreira acetylide addition. Further routine steps led to the sulfones 29 and 45, respectively. After merging them with 2‐bromobenzaldehyde 9 in a Julia–Kocienski reaction, metalation, carboxylation, and protecting‐group manipulations gave the seco acids 35 and 49. By means of lactonization under Mitsunobu (alcohol activation) or Trost–Kita conditions (carboxyl activation), all four possible macrocyclic ketone stereoisomers were accessible. In all, considering various protecting‐group decorations, 16 analogues were obtained and tested for cytotoxicity (L929 mouse fibroblast cell line). Whereas most of the analogues were less active than zearalenone (IC50=9.4 μM), the resorcinol derivatives were comparable, with one stereoisomer (40 b) being slightly more active (IC50=6.6 μM). These results were also reflected in the binding assays to Hsp90 in which 40 b showed a dissociation constant (Kd) value of 130 nM.
Bimetallic Catalysis Involving Dipalladium(I) and Diruthenium(I) Complexes
Authors: Das Raj K. , Saha Biswajit , Rahaman S. M. Wahidur , Bera Jitendra K. 
Published: October 2010

AbstractDipalladium(I) and diruthenium(I) compounds bridged by two [{(5,7‐dimethyl‐1,8‐naphthyridin‐2‐yl)amino}carbonyl]ferrocene (L) ligands have been synthesized. The X‐ray structures of [Pd2L2][BF4]2 (1) and [Ru2L2(CO)4][BF4]2 (2) reveal dinuclear structures with short metal–metal distances. In both of these structures, naphthyridine bridges the dimetal unit, and the site trans to the metal–metal bond is occupied by weakly coordinating oxygen from the amido fragment. The catalytic utilities of these bimetallic compounds are evaluated. Compound 1 is an excellent catalyst for phosphine‐free, Suzuki cross‐coupling reactions of aryl bromides with arylboronic acids and provides high yields in short reaction times. Compound 1 is also found to be catalytically active for aryl chlorides, although the corresponding yields are lower. A bimetallic mechanism is proposed, which involves the oxidative addition of aryl bromide across the PdPd bond and the bimetallic reductive elimination of the product. Compound 1 is also an efficient catalyst for the Heck cross‐coupling of aryl bromides with styrenes. The mechanism for aldehyde olefination with ethyl diazoacetate (EDA) and PPh3, catalyzed by 2, has been fully elucidated. It is demonstrated that 2 catalyzes the formation of phosphorane utilizing EDA and PPh3, which subsequently reacts with aldehyde to produce a new olefin and phosphine oxide. The efficacy of bimetallic complexes in catalytic organic transformations is illustrated in this work.
Ostensible Enzyme Promiscuity: Alkene Cleavage by Peroxidases
Authors: Mutti Francesco G. , Lara Miguel , Kroutil Markus , Kroutil Wolfgang 
Published: October 2010

AbstractEnzyme promiscuity is generally accepted as the ability of an enzyme to catalyse alternate chemical reactions besides the ‘natural’ one. In this paper peroxidases were shown to catalyse the cleavage of a CC double bond adjacent to an aromatic moiety for selected substrates at the expense of molecular oxygen at an acidic pH. It was clearly shown that the reaction occurs due to the presence of the enzyme; furthermore, the reactivity was clearly linked to the hemin moiety of the peroxidase. Comparison of the transformations catalysed by peroxidase and by hemin chloride revealed that these two reactions proceed equally fast; additional experiments confirmed that the peptide backbone was not obligatory for the reaction and only a single functional group of the enzyme was required, namely in this case the prosthetic group (hemin). Consequently, we propose to define such a promiscuous activity as ‘ostensible enzyme promiscuity’. Thus, we call an activity that is catalysed by an enzyme ‘ostensible enzyme promiscuity’ if the reactivity can be tracked back to a single catalytic site, which on its own can already perform the reaction equally well in the absence of the peptide backbone.
Treatment of epilepsy in children with developmental disabilities
Authors: Depositario‐Cabacar Dewi Frances T. , Zelleke Tesfaye‐Getaneh 
Published: October 2010

AbstractChildren with developmental disabilities are at increased risk for epilepsy with a prevalence rate higher than the general population. Some of the more common developmental disorders in childhood and the features of epilepsy in these conditions are discussed. Specifically, autism, cerebral palsy, mental retardation, and attention deficit and hyperactivity disorder are reviewed. Ideal treatment for developmentally‐disabled children with epilepsy entails maximal seizure control without any significant adverse effects from the anti‐epileptic drugs and good quality of life. Antiepileptic drugs' cognitive and behavioral adverse effects tend to occur more frequently in these children. Careful selection of the appropriate medication and close monitoring for drug adverse effects is important. The specific adverse effects of the older and newer antiepileptic drugs are also reviewed. © 2010 Wiley‐Liss, Inc. Dev Disabil Res Rev 2010;16:239–247.
Managing irritability and aggression in autism spectrum disorders in children and adolescents
Authors: Robb Adelaide S. 
Published: October 2010

AbstractChildren with autism and autism spectrum disorders have a high rate of irritability and aggressive symptoms. In one study up to 20% of children with autism have symptoms of irritability and aggression including aggression, severe tantrums, and deliberate self injurious behavior (Lecavalier [ 2006] J. Autism Dev. Disord. 36:1101–1114.). These symptoms can lead to impairment and distress in both home and school settings. Medications to treat the irritability will be discussed across categories of antipsychotics, antidepressants, antihypertensive agents, and others. Emphasis will be placed on medications with the most safety and efficacy and FDA approval. © 2010 Wiley‐Liss, Inc. Dev Disabil Res Rev 2010;16:258–264.
Review of pharmacotherapy options for the treatment of attention‐deficit/hyperactivity disorder (ADHD) and ADHD‐like symptoms in children and adolescents with developmental disorders
Authors: Rowles Brieana M. , Findling Robert L. 
Published: October 2010

AbstractDevelopmental disorders such as subaverage intelligence, pervasive developmental disorders, and genetic syndromes are frequently associated with comorbid attention‐deficit/hyperactivity disorder (ADHD) or ADHD‐like symptoms. While there are not pharmacological cures for these developmental disorders, coinciding ADHD and ADHD‐like symptoms that contribute to difficulties in psychosocial functioning are frequently able to be addressed by pharmacotherapy. This article reviews what is known about the efficacy and tolerability of pharmacological interventions for the treatment of children and adolescents suffering from developmental disorders and comorbid ADHD/ADHD‐like symptoms. © 2010 Wiley‐Liss, Inc. Dev Disabil Res Rev 2010;16:273–282.
Construction of Metal‐Organic Frameworks with the Pyridine‐3,5‐dicarboxylate Anion and Bis(imidazole) Ligands: Synthesis, Structure, and Thermostability Studies
Authors: Lv Yanyan , Qi Yan , Sun Lixin , Luo Feng , Che Yunxia , Zheng Jimin 
Published: October 2010

AbstractThree novel metal‐organic polymers, namely, [Ni(L1)0.5(3,5‐pdc)(μ‐H2O)]·H2O (1), [Co(L2)(3,5‐pdc)]·2H2O (2), and [Co(L3)(3,5‐pdc)(μ‐H2O)]·H2O (3) [3,5‐pdc = pyridine‐3,5‐dicarboxylate, L1 = 1,4‐bis(imidazol‐1‐ylmethyl)benzene, L2 = 1,1′‐(1,4‐butanediyl)bis(imidazole), and L3 = 1,1′‐(1,4‐hexanediyl)bis(imidazole)], have been hydrothermally synthesized and characterized by single‐crystal X‐ray diffraction (XRD), thermogravimetric (TG) analysis, and elemental analysis (EA). Polymers 1 and 2 have the identical 2D [M(3,5‐pdc)] sheet skeleton, however, different bis(imidazole) ligands cause 1 to have a 2D (3,4)‐connected double‐layer net featuring the (63)(64·82) topology symbol and 2 to have a 2D (3,5)‐connected net characterized by the (3 52)(32·53·64·7) topology symbol. They are both extended to 3D supramolecular frameworks through the O–H···O hydrogen bonds. In particular, in 2 there exists a four‐membered Z‐shape water cluster. The Co ions in 3 are connected by 3,5‐pdc and L3 to give rise to a complicated 3D framework that can be described as a (3,5)‐connected (42·5)(42·63·82·102·12) network. In addition, the thermal analysis of these three complexes has been measured and discussed.