bluuebunny/crossref_metadata_2025_split huggingface dataset:
Terlipressin: Vasopressin Analog and Novel Drug for Septic Shock
Authors: Pesaturo Adam B , Jennings Heath R , Voils Stacy A 
Published: December 2006

Objective:To review and assess available literature on chemistry, pharmacology, pharmacodynamics, pharmacokinetics, clinical studies, adverse events, drug interactions, and dosing and administration of terlipressin in septic shock.Data Sources:A literature search of MEDLINE (1966–September 2006), International Pharmaceutical Abstracts (1970–September 2006), and Cochrane database (third quarter 2006) was conducted, using key terms of terlipressin, lypressin, triglycyl-lysine vasopressin, hemodynamic support, septic shock, vasopressor, and V1 receptor agonist. Bibliographies of relevant articles were reviewed for additional references.Study Selection and Data Extraction:Available English-language literature, including abstracts, animal studies, preclinical studies, clinical trials, and review articles, were examined.Data Synthesis:Because of potentially favorable pharmacokinetics versus vasopressin and limited availability of vasopressin in some countries, the effects of terlipressin, a vasopressin analog, have been studied recently for the treatment of septic shock. When administered as a 1–2 mg intravenous dose in patients with septic shock, terlipressin increases mean arterial pressure, urine output, systemic vascular resistance index, pulmonary vascular resistance index, and left and right ventricular stroke work index while decreasing heart rate, cardiac output, lactate, and oxygen delivery and consumption index. It is unclear whether lower doses of terlipressin would produce a similar vasopressor response with fewer cardiopulmonary effects and whether the effects of the drug on oxygen transport indices are detrimental.Conclusions:Terlipressin is a promising investigational medication for treatment of septic shock. Small trials have shown terlipressin to have favorable effects on hemodynamics in patients with septic shock refractory to conventional vasopressor treatment. It should be used with extreme caution in patients with underlying cardiac or pulmonary dysfunction. Further studies are needed to verify safety, efficacy, and dosing of terlipressin in patients with septic shock, and its use cannot be recommended in lieu of vasopressin at this time.
Mutant huntingtin Impairs the Post-Golgi Trafficking of Brain-Derived Neurotrophic Factor But Not Its Val66Met Polymorphism
Authors: del Toro Daniel , Canals Josep M. , Ginés Silvia , Kojima Masami , Egea Gustavo , Alberch Jordi 
Published: December 2006

Brain-derived neurotrophic factor (BDNF) polymorphism is associated with the pathophysiology of several neurodegenerative disorders, including Huntington's disease. In view of these data and the involvement of huntingtin in intracellular trafficking, we examined the intracellular transport and release of Val66Val BDNF (Val-BDNF) and Val66Met BDNF (Met-BDNF) in transfected striatal knock-in cells expressing wild-type or mutant full-length huntingtin. Colocalization studies with specific markers for endoplasmic reticulum showed no differences between the Val-BDNF and Met-BDNF and were not modified by mutant huntingtin. However, post-Golgi trafficking was altered by mutant huntingtin dependent on the BDNF form. Thus, fluorescence recovery after photobleaching (FRAP) and inverse FRAP analysis showed retention of Met-BDNF in the Golgi apparatus with respect to Val-BDNF in wild-type cells. Strikingly, mutant huntingtin diminished post-Golgi trafficking of Val-BDNF, whereas Met-BDNF was not modified. Accordingly, a reduction in the number of transport vesicles was only observed in mutant huntingtin cells transfected with Val-BDNF but not Met-BDNF. Moreover, mutant huntingtin severely affected the KCl-evoked release of Val-BDNF, although it had little effect on Met-BDNF regulated release. The constitutive release of Val-BDNF or Met-BDNF in mutant cells was only slightly reduced. Interestingly, mutant huntingtin only perturbed post-Golgi trafficking of proteins that follow the regulated secretory pathway (epidermal growth factor receptor or atrial natriuretic factor), whereas it did not change those that follow the constitutive pathway (p75NTR).We conclude that mutant huntingtin differently affects intracellular transport and release of Val-BDNF and Met-BDNF. In addition, our findings reveal a new role for huntingtin in the regulation of the post-Golgi trafficking of the regulated secretory pathway.
Authentic Literacy Activities for Developing Comprehension and Writing
Authors: Duke Nell K. , Purcell‐Gates Victoria , Hall Leigh A. , Tower Cathy 
Published: December 2006

Authentic literacy activities in the classroom replicate and reflect literacy activities that occur in people's lives outside of school and instructional contexts. A growing body of research supports use of such activities in teaching and learning.The authors elaborate on the definition of authentic literacy, describe supporting research and theory, and give examples of authentic literacy activities documented in a research study. They identify strategies teachers can use to implement these activities for reading and writing, focusing particularly on science instruction.
Mind Magnets
Authors: Fortenberry Callie L. , Fowler Teri W. 
Published: December 2006

Mind magnets are maps to guide instruction and facilitate the comprehension processes. They extend individual comprehension strategy instruction, which does not typically show students how to link application of appropriate strategies to whole texts.The mind magnet framework allows teachers to plan powerful interactions between the reader and the text to maximize comprehension. A mind magnet is not a strategy but rather a technique used to activate the metacognitive processes of knowing how and when to apply a strategy to enhance comprehension. Mind magnets employ before‐, during‐, and after‐reading activities designed to attract the reader's thoughts to elements of text critical for meaning construction.
Revisiting the Role of Miscue Analysis in Effective Teaching
Authors: McKenna Michael C. , Picard Michelle Cournoyer 
Published: December 2006

Miscue analysis has been used by teachers and reading specialists for more than 30 years. Its purpose is to reveal strengths and weaknesses in how children process text and thereby inform instruction for individual learners. But is it still a useful approach?The authors assert that miscue analysis can indeed be a useful tool, though not for all the reasons its advocates have asserted over the years. Instead, educators should use miscue analysis more narrowly to monitor children's use of particular strategies during reading, particularly as they move from context dependency to automatic decoding.
High‐Poverty Schools That Beat the Odds
Authors: Cunningham Patricia M. 
Published: December 2006

Poverty is a strong predictor of lack of academic success in reading, and schools serving high‐poverty populations are more likely to show weak performance in high‐stakes tests. But some schools manage to beat these odds, with students outperforming peers from more advantaged neighborhoods. Why?The author showcases six high‐poverty schools where large numbers of children pass state tests. Teachers indicated that the most important factors influencing their schools' success are the amount of instruction provided, time devoted to real reading and writing, perseverance and persistence, and high levels of student engagement.
Importance of complex traits
Authors: Lee Joseph H. 
Published: December 2006

Abstract
Genetic studies of complex traits have yielded limited success. Yet it is important to study these complex traits since they represent major public health burden. Here I discuss the reasons for the difficulties and provide some successes as examples. Although no single approach is optimal for all traits, family‐based linkage methods followed by association methods have been most successful in identifying susceptibility genes for complex traits. With use of quantitative traits, these studies are designed to identify susceptibility genes that confer smaller effects. Large population‐based epidemiologic studies, on the other hand, are better suited for characterization of genes. In sum, it is important to choose an optimal study design for the hypothesis of interest and apply multiple integrated approaches.
Functional annotation of the mouse genome: the challenge of phenotyping
Authors: Brown Steve D. M. 
Published: December 2006

Abstract
Deciphering the function of all the genes in the human genome represents a major challenge in functional genomics for the twentyfirst century. The mouse is a key model organism in this endeavour. Programs are underway to generate mutations for every gene in the mouse genome. Determining the phenotype of each of these mouse mutants will be critical to the functional annotation of the mammalian genome and is dependent upon developments in the standardization and sophistication of phenotyping platforms as well as improvements in phenotype data tools.
Terminal osseous dysplasia with pigmentary defects: Clinical description of a new family
Authors: Baroncini Anna , Castelluccio Pia , Morleo Manuela , Soli Fiorenza , Franco Brunella 
Published: December 2006

AbstractTerminal osseous dysplasia with pigmentary defects is an extremely rare condition characterized by the triad of pigmentary anomalies of the skin, skeletal abnormalities of the limbs and recurring digital fibromatosis of childhood, with considerable interfamilial and intrafamilial variability of expression. It has recently been added to the small group of X‐linked dominant disorder with prenatal male lethality on the basis of a four‐generation pedigree in which only females were affected, male progeny was decreased and the number of spontaneous abortions was increased. In this clinical report, we describe a 2‐year‐old girl with full expression of the syndrome including skin defects, skeletal anomalies and recurrent fibromatosis of fingers and toes and her mother who presents with only multiple hypertrophic oral frenula. As previously demonstrated, our patients also show an extremely skewed X‐inactivation on blood cells, strongly suggesting that there is selective disadvantage for cells carrying the mutated gene on their active X chromosome. Terminal osseous dysplasia with pigmentary defects could represent an additional example of extreme intrafamilial variability as already described for other X‐linked dominant disorders. © 2006 Wiley‐Liss, Inc.
Neuropathic features in fragile X premutation carriers
Authors: Berry‐Kravis Elizabeth , Goetz Christopher G. , Leehey Maureen A. , Hagerman Randi J. , Zhang Lin , Li Lexin , Nguyen Danh , Hall Deborah A. , Tartaglia Nicole , Cogswell Jennifer , Tassone Flora , Hagerman Paul J. 
Published: December 2006

AbstractFragile X‐associated tremor/ataxia syndrome (FXTAS) is a progressive neurological condition occurring in fragile X premutation carriers, predominantly males, and resulting in CNS dysfunction including tremor, ataxia, Parkinsonism, and cognitive decline. Neuropathic signs have also been described. The objective of this study was to compare neuropathic signs in fragile X premutation carriers versus controls and determine the relationship of these signs to CGG repeat length and tremor/ataxia. A neuropathy scale was utilized to compare distal tendon reflexes and vibration sense in subjects from a large cohort of carriers and controls undergoing neurological exam and structured videotaping sessions for movement disorder rating. The male carrier group displayed more impairment on total neuropathy, vibration and reflex scores than the corresponding control group, while female carriers were not significantly different from controls. In males, after correction for age effects, there was a correlation between CGG repeat length and both total neuropathy and reflex impairments. Age‐adjusted partial correlation analyses showed an association between neuropathy scores and severity of ataxia but not tremor in carrier males and females. These data suggest that neuropathic signs are associated with the fragile X premutation, presumably occurring through the same mechanism proposed for CNS disease, namely, toxicity from expanded‐CGG‐repeat FMR1 mRNA. © 2006 Wiley‐Liss, Inc.
Unusually severe expression of craniofacial features in Aarskog‐Scott syndrome due to a novel truncating mutation of the FGD1 gene
Authors: Orrico A. , Galli L. , Obregon M.G. , de Castro Perez M.F. , Falciani M. , Sorrentino V. 
Published: December 2006

AbstractAarskog‐Scott syndrome (AAS) is a rare, clinically and genetically heterogeneous condition characterized by facial dysmorphic features, short stature, brachydactyly, and genital anomalies. The X‐linked form is caused by mutations of the FGD1 gene. Although clinical manifestations and diagnostic criteria are well established, diagnosis is not simple, as the spectrum of phenotypical features may be extremely variable. Here, we report on the clinical and genetic characterization of a family in which molecular analyses revealed the inheritance of a novel truncating mutation of the FDG1 gene (c.945insC) in two affected brothers, with one of them displaying unusually severe craniofacial abnormalities. This previously unreported combination of anomalies might be due to the occurrence of two distinct disorders (AAS and hemifacial microsomia) or may represent an extension of the AAS phenotypic spectrum. Our findings highlight the phenotypic heterogeneity of AAS, supporting the opinion that the FGD1 mutations result in a broad spectrum of severity and, in some cases, may express a clinical appearance very different than typically described. © 2006 Wiley‐Liss, Inc.
Two novel point mutations in the long‐range SHH enhancer in three families with triphalangeal thumb and preaxial polydactyly
Authors: Gurnett Christina A. , Bowcock Anne M. , Dietz Frederick R. , Morcuende Jose A. , Murray Jeffrey C. , Dobbs Matthew B. 
Published: December 2006

AbstractSpatio‐temporal expression of sonic hedgehog (SHH) is driven by a regulatory element (ZRS) that lies 1 Mb upstream from SHH. Point mutations within the highly conserved ZRS have been described in the hemimelic extra toes mouse and in four families with preaxial polydactyly [Lettice et al., 2003]. Four North American Caucasian families were identified with autosomal dominant triphalangeal thumb. DNA from 20 affected and 36 unaffected family members was evaluated by sequence analysis of a 774‐bp highly conserved ZRS contained within LMBR1 intron 5. Mutations within ZRS were identified in three of four families. In pedigree A and C, a novel A/G transition was identified near the 5′ end of ZRS at bp 739 that segregated with disease or carrier status. Pedigree A, described previously [Dobbs et al., 2000], is a large family with 19 affected members who exhibit a milder phenotype, including predominantly triphalangeal thumbs and low penetrance (82%) relative to other families. Pedigree C is a small family with two affected family members with triphalangeal thumb, and one affected with both triphalangeal thumb and preaxial polydactyly. A novel C/G mutation at bp 621 was identified in pedigree B that segregated with the disease in all four affected individuals who manifested both preaxial polydactyly and triphalangeal thumb. Both mutations alter putative Cdx transcription factor binding sites. Mutations within ZRS appear to be a common cause of familial triphalangeal thumb and preaxial polydactyly. A genotype/phenotype correlate is suggested by pedigree A, whose mutation lies near the 5′ end of ZRS; this family demonstrates a higher rate of nonpenetrance and milder phenotype. However, modifier genes may be contributing to the milder phenotype in this family. © 2006 Wiley‐Liss, Inc.
Anophthalmia‐plus syndrome: A clinical report and review of the literature
Authors: Makhoul Imad R. , Soudack Michalle , Kochavi Orna , Guilburd Joseph N. , Maimon Shimon , Gershoni‐Baruch Ruth 
Published: December 2006

AbstractWe describe a term male infant of healthy non‐consanguineous parents, born with congenital malformations, including bilateral cleft palate and lip, mild microphthalmia with iris coloboma and glaucoma of the right eye, and blepharophimosis with severe microphthalmia of the left eye. Spine radiograph and MRI showed first sacral hemivertebra with spina bifida, and agenesis of the 2nd, 3rd, 4th, and 5th sacral vertebrae and coccyx. Spine MRI showed caudal tethering of spinal cord at L3 level, filum terminalis lipoma and a syringomyelia. Brain ultrasound and MRI showed hypoplasia of corpus callosum with mild dilatation of the lateral ventricles. Orbital MRI showed bilateral microphthalmia—distorted small left eyeball with posteriorly located lens, and a split vitreous body in the right eye, suggestive of primary hyperplastic vitreous. The karyotype was normal. Summary of the findings in nine cases (our case and eight published cases) support the notion that anophthalmia‐plus syndrome (APS) is a distinct syndrome. Gene locus of APS is yet to be identified. © 2006 Wiley‐Liss, Inc.
Synthesis and Antiviral Evaluation of Some Sugar Arylglycinoylhydrazones and Their Oxadiazoline Derivatives
Authors: Abdel‐Aal Mohammed T. , El‐Sayed Wael A. , El‐Ashry El‐Sayed H. 
Published: December 2006

AbstractSugar N‐arylaminoacetylhydrazones 2–5 were prepared by the reaction of N‐arylaminoacetylhydrazides 1 with equivalent amounts of the corresponding monosaccharides. Per‐O‐acetyl derivatives 6‐9 of sugar hydrazones 2–5 were prepared by using acetic anhydride in pyridine at room temperature, while on boiling with acetic anhydride, cyclization had taken place to give the oxadiazolines 10–12. The prepared compounds were tested for antiviral activity against Herpes Simplex virus type‐1 (HSV‐1) and hepatitis‐A virus (HAV, MBB‐cell culture adapted strain). Plaque reduction infectivity assay was used to determine virus count reduction as a result of treatment with tested compounds.
Molecular portrait of high productivity in recombinant NS0 cells
Authors: Seth Gargi , Philp Robin J. , Lau Ally , Jiun Kok Yee , Yap Miranda , Hu Wei‐Shou 
Published: December 2006

AbstractMany important therapeutic proteins are produced in recombinant mammalian cells. Upon the introduction of the product gene, the isolated clones typically exhibit a wide range of productivity and high producers are subsequently selected for use in production. Using DNA microarray, two‐dimensional gel electrophoresis (2DE), and iTRAQ™ as global surveying tools, we examined the transcriptome and proteome profiles of 11 lines of NS0 cells producing the same antibody molecule. Genes that are significantly differentially expressed between high and low producer groups statistically fall into a number of functional classes. Their distribution among the functional classes differs somewhat between transcriptomic and proteomic results. Overall, a high degree of consistency between transcriptome and proteome analysis are seen, although some genes exhibiting inconsistent trends between transcript and protein levels were observed as expected. In a novel approach, functional gene networks were retrieved using computational pathway analysis tools and their association with productivity was tested by physiological comprehension of the possible pathways involved in high recombinant protein production. Network analysis indicates that protein synthesis pathways were altered in high producers at both transcriptome and proteome levels, whereas the effect on cell growth/death pathways was more prominent only at the transcript level. The results suggest a common mechanism entailing the alteration of protein synthesis and cell growth control networks leading to high productivity. However, alternate routes with different sets of genes may be invoked to give rise to the same mechanistic outcomes. Such systematic approaches, combining transcriptomic and proteomic tools to examine high and low producers of recombinant mammalian cells will greatly enhance our capability to rationally design high producer cells. This work is a first step towards shedding a new light on the global physiological landscape of hyper productivity of recombinant cells. Biotechnol. Bioeng. 2007;97: 933–951. © 2006 Wiley Periodicals, Inc.
Perfusion flow bioreactor for 3D in situ imaging: Investigating cell/biomaterials interactions
Authors: Stephens J.S. , Cooper J.A. , Phelan F.R. , Dunkers J.P. 
Published: December 2006

AbstractThe capability to image real time cell/material interactions in a three‐dimensional (3D) culture environment will aid in the advancement of tissue engineering. This paper describes a perfusion flow bioreactor designed to hold tissue engineering scaffolds and allow for in situ imaging using an upright microscope. The bioreactor can hold a scaffold of desirable thickness for implantation (>2 mm). Coupling 3D culture and perfusion flow leads to the creation of a more biomimetic environment. We examined the ability of the bioreactor to maintain cell viability outside of an incubator environment (temperature and pH stability), investigated the flow features of the system (flow induced shear stress), and determined the image quality in order to perform time‐lapsed imaging of two‐dimensional (2D) and 3D cell culture. In situ imaging was performed on 2D and 3D, culture samples and cell viability was measured under perfusion flow (2.5 mL/min, 0.016 Pa). The visualization of cell response to their environment, in real time, will help to further elucidate the influences of biomaterial surface features, scaffold architectures, and the influence of flow induced shear on cell response and growth of new tissue. Biotechnol. Bioeng. 2007;97: 952–961. © 2006 Wiley Periodicals, Inc.
Proteome analysis of heat shock protein expression in Pseudomonas alcaligenes NCIMB 9867 in response to gentisate exposure and elevated growth temperature
Authors: Zhao Bing , Yeo Chew Chieng , Tan Chew Ling , Poh Chit Laa 
Published: December 2006

AbstractPseudomonas alcaligenes NCIMB 9867 (strain P25X) degrades xylenols and cresols via the gentisate pathway. P25X expresses two isofunctional gentisate 1,2‐dioxygenases (GDO I and GDO II). The expression of both GDOs was not detected when P25X cells were grown at 42°C, even in the presence of gentisate. A total of 19 heat shock proteins (Hsps) belonging to the Hsp100, Hsp90, Hsp70, Hsp60, Hsp45, and small heat shock protein (sHsp) families were identified among the protein spots that were either newly detected or were expressed at levels of at least twofold higher when P25X cells were cultured at 32 or 42°C in the presence and absence of gentisate. Among these, 16 Hsps were commonly expressed at 42°C. Two additional Hsps (H5 and H13) from the Hsp90 and Hsp60 families, respectively, were expressed only when P25X cells were grown at 42°C and in the presence of gentisate. A protein of the sHsp (H16) family was expressed only in the presence of gentisate at 32°C but not at 42°C. The GroEL chaperonins of the Hsp60 family comprised the largest group of Hsps identified and exhibited high level of expression at 42°C following gentisate exposure. Biotechnol. Bioeng. 2007;97: 506–514. © 2006 Wiley Periodicals, Inc.