bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cerebrovasculopathy in NF1 associated with ocular and scalp defects
Authors: Smith Matt , Heran Manraj K.S. , Connolly Mary B. , Heran Harindar K. , Friedman J.M. , Jett Kimberly , Lyons Christopher J. , Steinbok Paul , Armstrong Linlea 
Published: December 2010

AbstractVascular lesions are uncommon in children with neurofibromatosis 1 (NF1) but can cause serious complications. We report on a child with NF1 who presented at 18 months of age with symptomatic stenosis of the left middle cerebral artery and its branches, and associated moyamoya disease. She also had bilateral posterior embryotoxon, left corneal opacity (Peters anomaly), and cutis aplasia of the left scalp. All of these defects may have occurred as a result of disruption of the blood supply caused by NF1 vasculopathy prenatally. This constellation of vascular anomalies has not been previously reported in a patient with NF1. © 2010 Wiley‐Liss, Inc.
Al‐Awadi–Raas‐Rothschild (limb/pelvis/uterus–hypoplasia/aplasia) syndrome and WNT7A mutations: Genetic homogeneity and nosological delineation
Authors: Garavelli Livia , Wischmeijer Anita , Rosato Simonetta , Gelmini Chiara , Reverberi Sandro , Sassi Silvia , Ferrari Adriano , Mari Francesca , Zabel Bernhard , Lausch Ekkehart , Unger Sheila , Superti‐Furga Andrea 
Published: December 2010

AbstractThe Al‐Awadi–Raas‐Rothschild syndrome (AARRS; OMIM 276820) and the Fuhrmann syndrome (FS; OMIM 228930) are distinct limb malformation disorders comprising different degrees of limb aplasia or hypoplasia. In 2006, Woods et al. found different recessive WNT7A mutations in one family segregating the AARRS phenotype and in a second family with FS. To explain the common genetic basis for the two clinically distinct disorders, functional studies were done showing that partial loss of WNT7A function resulted in FS, while complete loss of WNT7A function resulted in the more severe phenotype of AARRS. In spite of the elucidation of the molecular basis of AARRS, there remains to this day considerable diagnostic confusion that has culminated in the lumping of Schinzel phocomelia syndrome with AARRS; however, this phocomelic limb defect is quite different in its clinical aspect and pathogenesis from the limb findings of AARRS. Here, we report on a child with the AARRS phenotype and homozygosity for a non‐conservative E72K mutation in WNT7A, underline the homogeneity of the WNT7A‐associated AARRS phenotype, and propose differential diagnostic criteria for the AARRS reflecting the roles of WNT7A in limb development. © 2010 Wiley‐Liss, Inc.
A patient with a de novo distal 22q11.2 microdeletion and anxiety disorder
Authors: Verhoeven Willem , Egger Jos , Brunner Han , de Leeuw Nicole 
Published: December 2010

AbstractWe report on a young female with normal intelligence evaluated for long‐term anxiety. Her history includes prematurity, neonatal feeding problems, surgical correction of congenital heart defects, recurrent upper airway and urinary tract infections, and delayed motor and developmental milestones. Physical examination disclosed small stature and minor dysmorphisms. Chromosome analysis, 22q11.2 FISH analysis, and subtelomeric MLPA testing did not detect any abnormalities. Genome wide SNP Array analysis showed a de novo deletion in 22q11.21q11.22, the so‐called distal 22q11 microdeletion that involves the MAPK1 gene. A diagnosis of panic disorder was made and the patient was successfully treated with a daily dose of 20 mg citalopram. To our knowledge, this is the first adolescent patient with a long history of complaints about anxiety and a distal 22q11 microdeletion. We speculate that genes from the deleted region, especially MAPK1, increase the neurobiological susceptibility to anxiety disorders that may be a part of the psychopathological phenotype of the distal 22q11.2 microdeletion syndrome. © 2010 Wiley‐Liss, Inc.
The nosology of Richieri‐Costa/Guion‐Almeida syndrome(s)
Authors: Castori Marco , Cascone Piero , Brinelli Marco , Iannetti Giorgio , Grammatico Paola 
Published: December 2010

AbstractRichieri‐Costa/Guion‐Almeida syndrome type 1 (RCGAS1) is a rare MR/MCA syndrome comprising developmental and growth delay, microcephaly, prominent supraorbital ridges, asymmetric ptosis and eyebrows, esotropia, nystagmus, eye colobomas, and cleft lip/palate. It was originally described in three brothers and an additional sporadic male. The same authors also described a further family with a partially overlapping condition in two sisters (Richieri‐Costa/Guion‐Almeida syndrome type 2, RCGAS2). We report on a sporadic, mentally retarded patient strongly resembling those of the original report of RCGAS1, but lacking some manifestations such as short stature and, possibly, some ocular changes. Results of 200 kb CGH‐array study were normal. By comparing the present and previously reported patients with RCGAS1 and 2, we tried to contribute to syndrome delineation and to separate them from conditions with similar facial anomalies. © 2010 Wiley‐Liss, Inc.
Richieri‐Costa–Pereira syndrome: A unique acrofacial dysostosis type. An overview of the Brazilian cases
Authors: Favaro Francine Pinheiro , Zechi‐Ceide Roseli Maria , Alvarez Camila Wenceslau , Maximino Luciana P. , Antunes Luis Fernando B. B. , Richieri‐Costa Antonio , Guion‐Almeida Maria Leine 
Published: December 2010

AbstractWe reported on 16 new Brazilian patients and review findings in 12 previously reported cases (25 apparently unrelated Brazilian families) from Hospital of Rehabilitation of Craniofacial Anomalies, presenting with Richieri‐Costa–Pereira syndrome. All patients display a unique pattern of anomalies consisting of microstomia, micrognathia, abnormal fusion of mandible, cleft palate/Robin sequence, absence of central lower incisors, minor ears anomalies, hypoplastic first ray, abnormal tibiae, hypoplastic halluces, and clubfeet. Learning disability was also a common finding. The sex‐ratio showed deviation toward to female (1.8F:1M). Recurrence in sibs was observed in nine instances and consanguinity in 11, supporting the hypothesis of autosomal recessive inheritance. Nineteen of the 25 families lived in São Paulo State, seven of them (10 affected individuals) from an isolated region named “Vale do Ribeira.” The geographic barrier of this region associated with the high incidence of the consanguineous matting suggested that this condition is caused by a rare mutation with a founder effect. With the exception of one patient in France, all known cases are of Brazilian origin. The causative gene of this rare syndrome remains unknown. © 2010 Wiley‐Liss, Inc.
Mitochondrial phylogeny of tamarins (Saguinus, Hoffmannsegg 1807) with taxonomic and biogeographic implications for theS. nigricollisspecies group
Authors: Matauschek Christian , Roos Christian , Heymann Eckhard W. 
Published: December 2010

AbstractTamarins of the genusSaguinus, subfamily Callitrichinae, represent one of the most diverse primate radiations. So far, about 35 taxa have been described, but detailed information about their taxonomy and phylogeny is still lacking. To further elucidate the phylogenetic relationships and the biogeographic history within the genus, and to contribute to a more reliable classification of its taxa, we sequenced the complete mitochondrial cytochromebgene and the hypervariable region I of the D‐loop. Therefore, we mainly used fecal samples from wild tamarins collected during two expeditions to the Peruvian Amazon, an area of high tamarin diversity. Our data suggest that the numerous taxa of theS. nigricollisspecies group are derived from a common ancestor that separated from the other representatives of the genus ∼10 mya. Most taxa of theS. nigricollisgroup form monophyletic clusters, which mainly originated in a single rapid radiation ∼2.9 mya.S. fuscicollisandS. nigricollisappear as polyphyletic taxa, but we could identify various clusters, which are mainly consistent with differences in coat coloration. We could confirm most of the existing taxa as distinct entities and suggest species status forfuscicollis, illigeri, lagonotus, leucogenys, nigricollis, nigrifrons,tripartitus, andweddelli. Our genetic data do not support a separate status formelanoleucusandgraellsi, but due to differences in fur coloration, we give them subspecies status. The species group most likely originated in western Amazonia and diversified during the decline of the Acre wetland and the formation of the Amazonian river system. Am J Phys Anthropol, 2011. © 2010 Wiley‐Liss, Inc.
Chemical Control of Stem Cell Fate and Developmental Potential
Authors: Lyssiotis Costas A. , Lairson Luke L. , Boitano Anthony E. , Wurdak Heiko , Zhu Shoutian , Schultz Peter G. 
Published: December 2010

AbstractPotential applications of stem cells in medicine range from their inclusion in disease modeling and drug discovery to cell transplantation and regenerative therapies. However, before this promise can be realized several obstacles must be overcome, including the control of stem cell differentiation, allogeneic rejection and limited cell availability. This will require an improved understanding of the mechanisms that govern stem cell potential and the development of robust methods to efficiently control their fate. Recently, a number of small molecules have been identified that can be used both in vitro and in vivo as tools to expand stem cells, direct their differentiation, or reprogram somatic cells to a more naive state. These molecules have provided a wealth of insights into the signaling and epigenetic mechanisms that regulate stem cell biology, and are already beginning to contribute to the development of effective treatments for tissue repair and regeneration.
Preliminary study on the utilization of lignin as filler in ultra‐violet (UV) curable system
Authors: Rozman H. D. , Koay E. L. , Tay G. S. 
Published: December 2010

AbstractIn this study, Alcell lignin was used as filler in aliphatic urethane acrylate ultra violet (UV) curable resin system. The coating system was UV cured in the presence of free radical photoinitiator and cationic photoinitiator. The results showed that those with free radical photoinitiator produced coating film with higher degree of curing than those with cationic photoinitiator. The result of iodine number determination also showed that the unsaturated groups present in the UV curable system had reduced. This indicated that radical polymerization had taken place in the curing process of UV curable resin incorporated with lignin. In addition, the lignin content did not influence the curing process of the coating film. As for the coating film, it showed that the mechanical properties of the film were significantly influenced by the lignin content. This phenomenon was contributed by inherent stiffness of the lignin. © 2010 Wiley Periodicals, Inc. J Appl Polym Sci, 2011
Effects of α‐amylase and glycerol levels on the composition optimization of poly(β‐hydroxybutyrate‐co‐valerate)/starch blended biodegradable resin analyzed with response surface methodology
Authors: Cheng Wen‐Yen , Peng Jinchyau , Lui Wai‐Bun 
Published: December 2010

AbstractResponse surface methodology was used to analyze the effects of the enzyme level (X2) and glycerol level (X1) on objective attributes [water solubility index (WSI), water absorption index (WAI), and maximum loading (Y3)] of a cornstarch/poly(β‐hydroxybutyrate‐co‐valerate) blended composite. A rotatable central composite design was used to develop models for the objective responses. The experiments were run at barrel temperatures of 160, 160, 165, and 165°C, with a screw speed of 40 rpm and complete feeding (filling ratio = 1). Responses were most affected by changes in X2 and to a lesser extent by X1. Individual contour plots of the different responses were overlaid, and regions meeting the optimal WSI of 8.73%, WAI of 3.94 g of gel/g of dry weight, and Y3 of 304.17 N were established at an X2 of 5.43 g and an X1 of 120.79 mL. These predicted values for the optimal process conditions were in good agreement with the experimental data. © 2010 Wiley Periodicals, Inc. J Appl Polym Sci, 2011
Characterization of the fracture properties of aragonite‐ and calcite‐filled poly(ε‐caprolactone) by the essential work of fracture method
Authors: Tuba Ferenc , Oláh László , Nagy Péter 
Published: December 2010

AbstractBiodegradable polymers, blends, and composites are often investigated during tissue engineering studies, but their fracture properties, which are important mechanical engineering characteristics, are often disregarded or wrongly treated. In this study, essential work of fracture tests were performed on calcium carbonate filled poly(ε‐caprolactone), a very ductile polymer, to determine the effects of different filler shapes (calcite spheres and aragonite whiskers), sizes, and contents on the fracture parameters. Increasing the filler content caused stability problems during crack propagation, and this influenced the self‐similarity of the load–displacement response and resulted in the yielding point being missed. Moreover, the yielding‐related essential work of fracture and the energy dissipating during yielding were found to be almost independent of the filler content and thus could be indicators of matrix–filler adhesion. A shape effect of aragonite whiskers appeared during stable crack propagation; the motion of the particles and the friction on their surface slightly increased the dissipated energy quantum and resulted in a more oriented molecular structure. © 2010 Wiley Periodicals, Inc. J Appl Polym Sci, 2011
Fabrication of polymer‐gadolinium (III) complex nanomicelle from poly(ethylene glycol)‐polysuccinimide conjugate and diethylenetriaminetetraacetic acid‐gadolinium as magnetic resonance imaging contrast agents
Authors: Zhang Wei‐Lu , Yong Da‐Wei , Huang Jin , Yu Jia‐Hui , Liu Shi‐Yuan , Fan Ming‐Xia 
Published: December 2010

AbstractMethoxy poly (ethylene glycol)‐graft‐α, β‐poly (aspartic acid) derivatives (mPEG‐g‐PAA‐N3) were synthesized by sequential ring‐opening reaction of polysuccinimide (PSI) with mPEG‐NH2 (MW: 2000 Da), and 1‐azido‐3‐aminopropane, respectively. Then N2‐(hex‐5‐yne)‐diethylenetriamine‐tetra‐t‐butylacetate (DTTA‐der) was conjugated to mPEG‐g‐PAA‐N3 by click cycloaddition. After deprotection of carboxylic groups, mPEG‐g‐PAA‐DTTA macromolecular ligands were obtained. MPEG‐g‐PAA‐(DTTA‐Gd) complex nanomicelles were fabricated from mPEG‐g‐PAA‐DTTA and Gadolinium chloride. The formation of nanomicelles was confirmed by fluorescence spectrophotoscopy and particle size measurements. It was found that all the nanomicelles showed spherical shapes with core‐shell structures and narrow size distributions. Their sizes ranged from 50 to 80 nm, suggesting their passive targeting potential to tumor tissue. With the increase of graft degree (GD) of mPEG, the sizes of mPEG‐g‐PAA‐(DTTA‐Gd) nanomicelles showed a tendency to decrease. Compared with gadopentetate dimeglumine (Gd‐DTPA), mPEG‐g‐PAA‐(DTTA‐Gd) nanomicelles showed essential decreased cytotoxicity to KB cell line and enhanced T1‐weighted signal intensity, especially at low concentration of gadolinium (III), suggesting their great potentials as magnetic resonance imaging contrast agents. © 2010 Wiley Periodicals, Inc. J Appl Polym Sci, 2011
Novel dynamic vulcanization of polyethylene and ozonolysed natural rubber blends: Effect of curing system and blending ratio
Authors: Utara S. , Boochathum P. 
Published: December 2010

AbstractDynamic vulcanization was studied in terms of the change in α‐relaxation temperatures of the LDPE matrix, morphology, and mechanical properties of LDPE/ozonolysed NR blends which were vulcanized at various blend ratios and with different curing systems, i.e., peroxide and sulfur systems. The ozonolysed NR with Mw = 8.30 × 105 g mol−1 and Mn = 2.62 × 105 g mol−1, prepared by the in situ ozonolysis reaction of natural rubber latex, was used in this study. The significant change in the α‐relaxation temperature of LDPE in the LDPE/ozonolysed NR, dynamically vulcanized using the sulfur system, suggested that sulfur vulcanization of the blend gave a higher degree of crosslink density than using peroxide and corresponded with the improved damping property and homogenous phase morphology. However, the peroxide cured blends of LDPE/ozonolyzed NR gave more improvement of tensile strength and elongation at break than the sulfur cured system. Furthermore, the mechanical properties of tensile strength, elongation at break, and damping were improved by increasing the ozonolyzed natural rubber content in both DCP and sulfur cured blends. © 2010 Wiley Periodicals, Inc. J Appl Polym Sci, 2011
Synthesis and characterization of grafted carboxymethyl guar gum
Authors: Adhikary P. , Krishnamoorthi S. , Singh R. P. 
Published: December 2010

AbstractGraft copolymers of carboxymethyl guar gum (CMGG) and polyacrylamide (PAM) have been synthesized by grafting polyacrylamide chains onto carboxymethyl guar gum backbone using a ceric‐ion‐induced solution polymerization technique. By varying the amount of initiator, three different grades of graft copolymers are synthesized. The characterization of graft copolymer is carried out by FTIR, NMR, intrinsic viscosity measurement, SEM, SLS, DTG, and rheology. A comparison of flocculation efficiency of CMGG and its graft copolymer shows that the graft copolymer exhibits better flocculation performance. © 2010 Wiley Periodicals, Inc. J Appl Polym Sci, 2011
Cure characteristics, swelling behaviors, and mechanical properties of carbon black filler reinforced EPDM/NBR blend system
Authors: Manoj K. C. , Kumari Prajitha , Unnikrishnan G. 
Published: December 2010

AbstractThe effect of carbon black fillers viz. semireinforcing furnace (SRF), high abrasion furnace (HAF) and intermediate super abrasion furnace (ISAF) carbon blacks on the cure, swelling and mechanical properties of 70/30 EPDM/NBR blend have been investigated. The maximum torque values have been found to be increased with increase in filler loading. Filledsystems have been found to exhibit a reduced solvent uptake tendency compared to the unfilled sample. Blends loaded with ISAF exhibited the lowest toluene uptake among the carbon black filled systems due to the better filler reinforcement. A more uniform morphology has been observed for ISAF‐filled samples compared to the other filler loaded systems. The improvement in the mechanical properties has been observed to be the highest for ISAF‐filled samples followed by HAF and SRF filled systems. This has been attributed to the smaller particle size of ISAF black. The experimental results of mechanical testing have been compared with various theoretical models. © 2010 Wiley Periodicals, Inc. J Appl Polym Sci, 2011
Effect of molecular weight and testing rate on peel and shear strength of epoxidized natural rubber (ENR 50)‐based adhesives
Authors: Khan Imran , Poh B. T. 
Published: December 2010

AbstractThe dependence of peel strength and shear strength of epoxidized natural rubber (ENR‐50)‐based pressure sensitive adhesive on molecular weight and rate of testing was investigated using coumarone‐indene as the tackifying resin. Toluene and polyethylene terephthalate were used as the solvent and substrate respectively, throughout the study. A SHEEN hand coater was used to coat the adhesive on the substrate at a coating thickness of 120 μm. All the adhesion properties were determined by a Llyod Adhesion Tester operating at different rates of testing. Result shows that peel strength and shear strength increases up to an optimum molecular weight of 4.2 × 104 of ENR 50. For peel strength, the observation is attributed to the combined effects of wettability and mechanical strength of rubber at the optimum molecular weight, whereas for the shear strength, it is ascribed to the optimum cohesive and adhesive strength which enhances the shear resistance of the adhesive. Peel strength and shear strength also increases with increase in rate of testing, an observation which is associated to the viscoeslastic response of the adhesive. DSC and FTIR study confirms the miscibility of tackifier and the ENR 50. © 2010 Wiley Periodicals, Inc. J Appl Polym Sci, 2011
Locations of the Motor Endplate Band and Motoneurons Innervating the Sternomastoid Muscle in the Rat
Authors: Zhang Xiaolin , Mu Liancai , Su Hungxi , Sobotka Stanislaw 
Published: December 2010

AbstractSternocleidomastoid (SCM) is a long muscle with two bellies, sternomastoid (SM) and cleidomastoid (CM) in the lateral side of the neck. It has been widely used as muscle and myocutaneous flap for reconstruction of oral cavity and facial defects and as a candidate for reinnervation studies. Therefore, exact neuroanatomy of the SCM is critical for guiding reinnervation procedures. In this study, SM in rats were investigated to document banding pattern of motor endplates (MEPs) using whole‐mount acetylcholinesterase (AChE) staining and to determine locations of the motoneurons innervating the muscle using retrograde horseradish peroxidase (HRP) tracing technique. The results showed that the MEPs in the SM and CM were organized into a single band which was located in the middle portion of the muscle. After HRP injections into the MEP band of the SM, ipsilaterally labeled motoneurons were identified in the caudal medulla oblongata (MO), C1, and C2. The SM motoneurons were found to form a single column in lower MO and dorsomedial (DM) nucleus in C1. In contrast, the labeled SM motoneurons in C2 formed either one (DM nucleus), two [DM and ventrolateral (VL) nuclei], or three [DM, VL, and ventromedial (VM)] columns. These findings are important not only for understanding the neural control of the muscle but also for evaluating the success rate of a given reinnervation procedure when the SM is chosen as a target muscle. Anat Rec, 2011. © 2010 Wiley‐Liss, Inc.
Allometric and Metameric Shape Variation in Pan Mandibular Molars: A Digital Morphometric Analysis
Authors: Singleton Michelle , Rosenberger Alfred L. , Robinson Chris , O'neill Rob 
Published: December 2010

AbstractThe predominance of molar teeth in fossil hominin assemblages makes the patterning of molar shape variation a topic of bioanthropological interest. Extant models are the principal basis for understanding dental variation in the fossil record. As the sister taxon to the hominin clade, Pan is one such model and the only widely accepted extant hominid model for both interspecific and intraspecific variation. To explore the contributions of allometric scaling and meristic variation to molar variation in Pan, we applied geometric shape analysis to 3D landmarks collected from virtual replicas of chimpanzee and bonobo mandibular molars. Multivariate statistical analysis and 3D visualization of metameric and allometric shape vectors were used to characterize shape differences and test the hypothesis that species of Pan share patterns of metameric variation and molar shape allometry. Procrustes‐based shape variables were found to effectively characterize crown shape, sorting molars into species and tooth‐row positions with ≥95% accuracy. Chimpanzees and bonobos share a common pattern of M1–M2 metameric variation, which is defined by differences in the relative position of the metaconid, size of the hypoconulid, curvature of the buccal wall, and proportions of the basins and foveae. Allometric scaling of molar shape is homogeneous for M1 and M2 within species, but bonobo and chimpanzee allometric vectors are significantly different. Nevertheless, the common allometric shape trend explains most molar‐shape differences between P. paniscus and P. troglodytes. When allometric effects are factored out, chimpanzee and bonobo molars are not morphometrically distinguishable. Implications for hominid taxonomy and dietary reconstruction are discussed. Anat Rec, 2011. © 2010 Wiley‐Liss, Inc.