bluuebunny/crossref_metadata_2025_split huggingface dataset:
Manifestation of diffuse yellowish keratoderma on the palms and soles in autosomal recessive congenital ichthyosis patients may be indicative of mutations in NIPAL4
Authors: ALAVI Afagh , SHAHSHAHANI Mostafa M. , KLOTZLE Brandy , FAN Jian‐Bing , RONAGHI Mostafa , ELAHI Elahe 
Published: November 2011

AbstractIchthyosis is a heterogeneous disorder characterized by abnormal skin scaling over the whole body. Autosomal recessive congenital ichthyosis (ARCI) comprises various forms, the most important of which are lamellar ichthyosis (LI) and congenital ichthyosiform erythroderma (CIE). Seven genes have been identified to be causative of ARCI, and these account for disease in 60–80% of the patients. There is notable phenotypic overlap between the major forms of ARCI, and a strong genotype–phenotype correlation has not been found. Here, we initially aimed to identify the causative gene in a large Iranian ARCI pedigree, and subsequently performed genetic analysis on four other affected pedigrees. A genotype–phenotype correlation was sought. Whole genome homozygosity mapping using high‐density single nucleotide polymorphism chips was performed on the large pedigree. Linkage to chromosome 5 and a mutation in NIPAL4 causing p.G297R were identified. The same mutation was also identified in two of the remaining four Iranian pedigrees. Two of the NIPAL4 mutation bearing pedigrees were classified as CIE and one as LI. Notably, all NIPAL4 mutation‐bearing patients manifested diffuse yellowish keratoderma on the palms and soles. We provide evidence suggesting presentation of this diffuse yellowish keratoderma may be indicative of mutations in NIPAL4, providing an easily assessable genotype–phenotype correlation.
Nestin‐positive hair follicle pluripotent stem cells can promote regeneration of impinged peripheral nerve injury
Authors: AMOH Yasuyuki , AKI Ryoichi , HAMADA Yuko , NIIYAMA Shiro , ESHIMA Koji , KAWAHARA Katsumasa , SATO Yuichi , TANI Yoichi , HOFFMAN Robert M. , KATSUOKA Kensei 
Published: November 2011

AbstractNestin‐positive, keratin 15 (K15)‐negative multipotent hair follicle stem cells are located above the hair follicle bulge. We have termed this location the hair follicle pluripotent stem cell area. We have previously shown that transplantation of nestin‐expressing hair follicle stem cells can regenerate peripheral nerve and spinal cord injuries. In the present study, we regenerated the impinged sciatic nerve by transplanting hair follicle pluripotent stem cells. Human hair follicle stem cells were transplanted around the impinged sciatic nerve of ICR nude (nu/nu) mice. The hair follicle stem cells were transplanted between impinged sciatic nerve fragments of the mouse where they differentiated into glial fibrillary acidic protein‐positive Schwann cells and promoted the recovery of pre‐existing axons. The regenerated sciatic nerve functionally recovered. These multipotent hair follicle stem cells thereby provide a potential accessible, autologous source of stem cells for regeneration therapy of nerves degenerated by compression between bony or other hard surfaces.
CD48 as a novel molecular target for antibody therapy in multiple myeloma
Authors: Hosen Naoki , Ichihara Hiroyoshi , Mugitani Atsuko , Aoyama Yasutaka , Fukuda Yuki , Kishida Satoshi , Matsuoka Yoshikazu , Nakajima Hiroko , Kawakami Manabu , Yamagami Tamotsu , Fuji Shigeo , Tamaki Hiroya , Nakao Takafumi , Nishida Sumiyuki , Tsuboi Akihiro , Iida Shinsuke , Hino Masayuki , Oka Yoshihiro , Oji Yusuke , Sugiyama Haruo 
Published: November 2011

SummaryMonoclonal antibody (mAb) drugs are desirable for the improvement of multiple myeloma (MM) treatment. In this study, we found for the first time that CD48 was highly expressed on MM plasma cells. In 22 out of 24 MM patients, CD48 was expressed on more than 90% of MM plasma cells at significantly higher levels than it was on normal lymphocytes and monocytes. CD48 was only weakly expressed on some CD34+ haematopoietic stem/progenitor cells, and not expressed on erythrocytes or platelets. We next examined whether CD48 could serve as a target antigen for mAb therapy against MM. A newly generated in‐house anti‐CD48 mAb induced mild antibody‐dependent cell‐mediated cytotoxicity and marked complement‐dependent cytotoxicity against not only MM cell lines but also primary MM plasma cells in vitro. Administration of the anti‐CD48 mAb significantly inhibited tumour growth in severe combined immunodeficient mice inoculated subcutaneously with MM cells. Furthermore, anti‐CD48 mAb treatment inhibited growth of MM cells transplanted directly into murine bone marrow. Finally and importantly, we demonstrated that the anti‐CD48 mAb did not damage normal CD34+ haematopoietic stem/progenitor cells. These results suggest that the anti‐CD48 mAb has the potential to become an effective therapeutic mAb against MM.
The autoreactivity of B cells in hereditary angioedema due to C1 inhibitor deficiency
Authors: Kessel A , Peri R , Perricone R , Guarino M D , Vadasz Z , Novak R , Haj T , Kivity S , Toubi E 
Published: November 2011

SummaryPatients with hereditary angioedema (HAE) tend to produce autoantibodies and have a propensity to develop immunoregulatory disorders. We characterize the profile of autoantibodies in a group of HAE patients and investigate their memory B cells' phenotype and activation status. We studied the activity status phenotype, Toll-like receptor (TLR)-9 expression and total phosphotyrosine in B cells isolated from HAE patients. Additionally, the following autoantibodies were assessed in the serum of 61 HAE patients: anti-nuclear, rheumatoid factor, anti-cardiolipin, anti-tissue transglutaminase, anti-endomysial, anti-Saccharomyces cerevisiae, anti-thyroid and anti-neutrophil cytoplasmic antibodies. In 47·5% of HAE patients we detected at least one of the tested autoantibodies. Expression of CD69, CD5 and CD21 was found to be significantly higher on memory B cells from HAE patients compared to healthy controls (4·59 ± 4·41 versus 2·06 ± 1·81, P = 0·04, 8·22 ± 7·17 versus 3·65 ± 3·78, P = 0·05, 2·43 ± 0·54 versus 1·92 ± 0·41, P = 0·01, respectively). Total phosphotyrosine in B cells from HAE patients was significantly higher compared to healthy controls (4·8 ± 1·1 versus 2·7 ± 1·3, P = 0·0003). Memory B cells isolated from the HAE group contained higher amounts of TLR-9 compared to healthy controls (8·17 ± 4·1 versus 4·56 ± 1·6, P = 0·0027). Furthermore, the expression of TLR-9 in memory B cells from HAE patients with autoantibodies was significantly higher than the control group (10 ± 4·7 versus 4·56 ± 1·6, P = 0·0002) and from that in HAE patients without autoantibodies (10 ± 4·7 versus 5·8 ± 0·9, P = 0·036). HAE patients have enhanced production of autoantibodies due most probably to the increased activation of B cells, which was found to be in association with a high expression of TLR-9.
Induction of elastin expression in vascular endothelial cells relates to hepatoportal sclerosis in idiopathic portal hypertension: possible link to serum anti-endothelial cell antibodies
Authors: Sato Y , Ren X S , Harada K , Sasaki M , Morikawa H , Shiomi S , Honda M , Kaneko S , Nakanuma Y 
Published: November 2011

SummaryHepatoportal sclerosis accompanied by dense elastic fibre deposition is generally regarded as the primary lesion in the development of idiopathic portal hypertension (IPH). This study was performed to clarify the mechanism of elastic fibre deposition in the peripheral portal tracts of IPH liver in relation to serum anti-endothelial cell antibodies (AECA). In-vitro experiments were performed using human dermal microvascular endothelial cells (HMVEC) and patients' sera. The presence of serum AECA was assayed by a cell-based enzyme-linked immunosorbent assay (ELISA) using HMVEC. Immunohistochemical analysis of elastin was performed using liver tissue sections of IPH patients. IPH sera contained one or more AECA that could bind to the vascular endothelial cells of the peripheral portal tracts of the liver. When the value of AECA greater than the mean ± 2 standard deviations of healthy controls was regarded as positive, the positive detection rate of either immunoglobulin (Ig)G, IgA or IgM AECA in IPH sera was 30% (10 of 33 cases). IPH sera induced the expression of elastin in HMVEC, which appeared to be associated with the presence of AECA. Apoptosis was also induced in HMVEC by the stimulation with IPH sera. In vivo, elastin expression was observed in the endothelial cells of the peripheral portal tracts of IPH livers in a proportion of cases. The disease pathogenesis of IPH seems to be heterogeneous, and this study elucidated a possible contribution of the induction of elastin expression in the portal vessels to hepatoportal sclerosis of IPH, which might be linked to serum AECA as a causative factor.
A comparative study on the polyphenolic content, antibacterial activity and antioxidant capacity of different solvent extracts of Brassica oleracea vegetables
Authors: Jaiswal Amit K. , Abu‐Ghannam Nissreen , Gupta Shilpi 
Published: November 2011

SummaryBrassica vegetables are rich in polyphenols, flavonoids and glucosinolates. Investigation was undertaken to optimise the best solvents among 60% ethanol, acetone and methanol for the extraction of polyphenols from Brassica vegetables. Furthermore, different properties such as antibacterial activity and antioxidant capacity were also investigated. Results showed that a 60% methanolic extract showed the highest total phenolic content which was 23.6, 20.4 and 18.7 mg gallic acid equivalents (GAE) g−1 extract for broccoli, Brussels sprouts and white cabbage, respectively. The hydroxybenzoic acid content of various solvent extracts ranged from 5.86 to 8.91 GAE g−1 extract for broccoli, 2.70 to 5.44 GAE g−1 extract for Brussels sprouts and 3.69 to 4.86 GAE g−1 extract for white cabbage, while the hydroxycinnamic acid content ranged from 0.78 to 1.26 chlorogenic acid equivalents (CAE) g−1 extract for broccoli, 1.41 to 3.45 CAE g−1 extract for Brussels sprouts and 0.49 to 1.14 CAE g−1 extract for white cabbage. A concentration‐dependent antioxidative capacity was confirmed for different reactive oxygen species, and moderate antibacterial activity was observed against a number of Gram‐negative and Gram‐positive food spoilage and food pathogenic bacteria. Solvents significantly affected polyphenolic content and its different properties, and the methanol was found to be the best solvent for the extraction of polyphenols from studied Brassica vegetables.
Mass transfer kinetics and regressional‐desirability optimisation during osmotic dehydration of pumpkin, kiwi and pear
Authors: Arballo Javier R. , Bambicha Ruth R. , Campañone Laura A. , Agnelli Miriam E. , Mascheroni Rodolfo H. 
Published: November 2011

SummaryMass transfer kinetics and optimisation of osmotic dehydration (OD) of fruits and vegetables with diverse structures were studied. Different concentrations of sucrose (20–60 °Brix) and process times (0–24 h) were used. Magee’s model was appropriate for predicting water loss (WL), while Azuara’s model fitted well solids gain (SG) data and represented more accurately the evolution of the complete process close to equilibrium. Polynomial equations for each kinetic variable [WL, SG and weight reduction (WR) – for pumpkin, kiwi and pear] using multiple linear regression were fitted for a selected range of experimental data (30–240 min, 20–60 °Brix). A complete solution algorithm for desirability function was coded in Matlab® 7.2 (Mathworks, Natick, MA, USA) with the aim to optimise osmotic dehydration process in terms of WL, SG and WR; optimal conditions were found for each fruit. Besides, an optimal common zone was identified for OD corresponding to process time from 114 to 240 min and sucrose concentration from 54 to 60 °Brix.
Effects of maltodextrin level, frying temperature and time on the moisture, oil and beta‐carotene contents of vacuum‐fried apricot slices
Authors: Diamante Lemuel M. , Savage Geoffrey P. , Vanhanen Leo , Ihns Reiner 
Published: November 2011

SummaryResponse surface methodology was used to investigate the effects of frying temperature, time and maltodextrin (MD) levels on the moisture, oil and beta‐carotene contents of the vacuum‐fried apricot slices. Based on the results, the moisture content of vacuum‐fried apricot slices decreases with increasing frying temperature and frying time. Generally, the oil content of vacuum‐fried apricot slices decreases with decreasing frying temperature and frying time. The beta‐carotene content of vacuum‐fried apricot slices increases with increasing frying temperature. But the trend of increase in the beta‐carotene content of the product with frying temperature was higher at lower MD level. When processing vacuum‐fried apricot slices, a frying temperature of 100 °C, frying time of 72.5 min and an MD level of 70% must be used to achieve a product of acceptable moisture, oil and beta‐carotene contents.
Comparative fruit quality parameters of several Japanese plum varieties in two newly established orchards, organic and conventionally managed
Authors: Daza Antonio , Camacho María , Galindo Irene , Arroyo Francisco T. , Casanova Laura , Santamaría Carmen 
Published: November 2011

SummaryThe fruit quality parameters of several Japanese plum cultivars (Prunus salicina Lindl) were studied in two experimental orchards under organic and conventional management. The study was performed during 2008–2010 in the province of Seville (SW Spain). Colour, weight, size, firmness, soluble solid concentration and acidity were measured for fruit quality evaluation. Yield was also determined. In general, the organic fruit was of smaller size and weight. There was little difference in the colour of most of the varieties, but some varieties such as ‘Friar’ and ‘Black Amber’ were more colourful in the organic orchard. The soluble solid concentration and acidity were similar in the fruit from both orchards. The organic plums showed firmness equal to or slightly greater than conventional ones. Dry matter and nitrites were analysed in 2010, and no significant differences were observed in most of the varieties. The accumulated fruit yield was significantly superior in the conventionally managed orchard.
Simple determination of formaldehyde in fermented foods by HS‐SPME‐GC/MS
Authors: Shin Ho‐Sang , Lim Hyun‐Hee 
Published: November 2011

SummaryWe describe an automatic method to detect formaldehyde (FA) in some fermented foods. This method is based on derivatisation with 2,2,2‐trifluoroethylhydrazine (TFEH) and consecutive headspace solid‐phase micro‐extraction and gas chromatography‐mass spectrometry. FA in food reacted for 30 min at 40 °C with 2,2,2‐TFEH in a headspace vial, and the formed FA‐TFEH derivative was simultaneously vaporised and adsorbed on 85‐μm carboxen–polydimethylsiloxane. Under the established condition, the limit of detection was 0.1 μg kg−1 by using 1.0‐g solid food and 1.0‐mL liquid food, and the relative standard deviation was less than 10% at FA concentrations between 0.050 and 0.500 mg kg−1. The concentrations of FA in several traditional Korean foods including gimchi, watery radish gimchi, soybean paste, red pepper paste, soy sauce and bean‐paste soup were measured. All food samples had detectable levels of FA (0.104–13.05 mg kg−1).
A preliminary study of monosaccharide composition and α‐glucosidase inhibitory effect of polysaccharides from pumpkin (Cucurbita moschata) fruit
Authors: Song Yi , Zhang Yuyu , Zhou Tingting , Zhang Hua , Hu Xiaosong , Li Quanhong 
Published: November 2011

SummaryIn this work, crude polysaccharide extracts were extracted from pumpkin (Cucurbita moschata) fruit by hot water. After removal of proteins and purification, polysaccharides of pumpkin fruit (PP1‐1) were subjected to structural identification. Gas chromatography analysis indicated that PP1‐1 comprised of galactose (86.4%), and glucose (13.6%). The molecular weight of PP1‐1 was measured to be 0.87 × 104 Da by gel permeation chromatography. The inhibitory kinetic evaluation showed that it was non‐competitive inhibition of PP1‐1 on the α‐glucosidase‐catalysed hydrolysis of PNPG. The Michaelis–Menten constant (Km) was 0.106 m, and the inhibitory constants (Ki), 0.435 mg.
Significance and extent of secondary seed dispersal by predatory birds on oceanic islands: the case of the Canary archipelago
Authors: Padilla David P. , González‐Castro Aarón , Nogales Manuel 
Published: November 2011

Summary1. Secondary seed dispersal is a multistep process with two or more phases, which involve different dispersers that usually extend the distance from the seed’s parent plant. This ecological process has been recorded in some subtropical oceanic islands, where predatory birds commonly consume frugivorous lizards and disperse seeds already consumed by the lizards.2. We evaluated the overall importance of this type of secondary dispersal in the Canary Islands, the only place worldwide where it has been studied in depth. From an examination of all the islands and their suitable habitats, we found seeds from 78 plant species inside 2098 shrike pellets and 5304 kestrel pellets. A greater number of species were secondarily dispersed by kestrels (76; 97%) than by shrikes (26; 34%).3. Forty‐four (56%) of the total species detected in pellets were identified at the species level, comprising 73% native and 27% introduced species. Seventy per cent of these identified species were fleshy fruit‐bearing plants and 84% of the interactions took place in open habitats, close to coastal areas.4. Germination experiments showed that seeds of at least 32 plant species were viable after being removed from the bird pellets. A similar pattern of seed germination was detected for seeds from the droppings of lizards and pellets of shrikes, showing both to be effective dispersers. However, the seeds dispersed by kestrels had different levels of success depending on the number of gut passages experienced. Seeds that had undergone double gut treatment (lizard and secondary ingestion by kestrel) had reduced germination rates of many small‐ and medium‐sized seeds compared with seeds ingested by lizards and discarded inside the lizard guts by kestrels.5. We also studied the relationship between body length and gape width of lizards in order to assess limitations on the sizes and quantities of seeds available for secondary dispersal. Kestrels can disperse a greater number and variety of seeds because they predate larger lizards that potentially carry greater seed loads.6. Synthesis. The current results show how these non‐standard long‐distance dispersal events produced by predatory birds can be considered as a regular and generalized process on all islands of the Canary archipelago.
Clinicians' views of formats of performance comparisons
Authors: Allwood Dominique , Hildon Zoe , Black Nick 
Published: November 2011

AbstractRationale, aims and objectives  Comparisons of the performance of health care providers are increasingly being used. Despite one key audience being clinicians, there has been little research on the format and content of such comparisons. Our aim was to explore clinicians' comprehension and preferences of format and content in displaying provider outcomes using comparisons of patient reported outcome measures data.Method  A qualitative study, based on seven meetings involving 107 clinicians (mostly consultant and junior doctors, and nurses), revealed their views on nine formats and five aspects of content.Results  Key findings were the desire for data in more than one format, explicit display of comparative performance (rank order) and the need for explanations (e.g. of unfamiliar formats and of statistical uncertainty).Conclusions  Several themes were identified that shaped clinicians' views. Results were sufficiently clear to permit recommendations for the form and content of standard reports for the National Health Service.
Rendering clinical psychology an evidence‐based scientific discipline: a case study
Authors: St. Stoyanov Drozdstoj , Machamer Peter K. , Schaffner Kenneth F. 
Published: November 2011

AbstractRationale, aims and objectives Both modern neuroscience and clinical psychology taken as separate fields have failed to reveal the explanatory mechanisms underlying mental disorders. The evidence acquired inside the mono‐disciplinary matrices of neurobiology, clinical psychology and psychopathology are deeply insufficient in terms of their validity, reliability and utility. Further, no effectivetrans‐disciplinary connections have been developed between them.ArgumentIn this context, our case study aims at illustrating some specific facets of clinical psychology as a crucial discipline for explaining and understanding mental disorder. The methods employed in clinical psychology are scrutinized using the exemplar case of the Minnesota Multiphasic Personality Inventory (MMPI). We demonstrate that a clinical interview and a clinical psychological rating scale consist of the same kind of cognitive content. The provisional difference can be described in terms of its having two comparable complementary cognitive structures. The test is composed of self‐evaluation reports (items) formulated as questions or statements. The psychopathological structured interview is formulated in terms of subjective experience indicated as symptoms (these are self‐reports recorded by the physician), complemented with the so‐called ‘signs’ or the presumably ‘objective’ observations of the overt behaviours of the patient. However, the cognitive content of clinical judgment is beyond any doubt as subjective as the narrative of the patient. None of the components of the structured psychopathological interview is independent of the inter‐subjective system created in the situation of clinical assessment.ConclusionTherefore, the protocols from various clinicians that serve to sustain the reliability claim of the ‘scientific’ Diagnostic Statistical Manual of Mental Disorders cannot be regarded as independent measurements of the cognitive content and value of the psychological rating scales or vice versa.
The pathology of ‘scale drop syndrome’ in Asian seabass, Lates calcarifer Bloch, a first description
Authors: Gibson‐Kueh S , Chee D , Chen J , Wang Y H , Tay S , Leong L N , Ng M L , Jones J B , Nicholls P K , Ferguson H W 
Published: November 2011

AbstractThis is the first pathological description of ‘scale drop syndrome’ (SDS) in Asian seabass, Lates calcarifer Bloch. Cumulative mortality was estimated at 40–50%. The vasculitis in all major organs including the skin and associated tissue necrosis was distinctive. The dermis overlying scale beds was often necrotic and associated with scale loss. Necrosis of splenic ellipsoids, renal glomeruli and choroid rete glands of eye were further hallmarks of a disease with systemic vascular involvement. The brain was not spared vascular damage, and the resulting multifocal encephalomalacia probably accounts for the spiral swimming behaviour in some affected fish. Other lesions included accentuated hepatic lobulation and gastric gland necrosis. Nuclear chromatin margination and karyolysis in hepatocytes, renal tubular epithelium and gastric and intestinal epithelium suggest specific targeting of cells. Basophilic cytoplasmic inclusions were present in spleen, kidney, liver, heart and choroid rete, but they were not prominent. Using transmission electron microscopy, two morphological forms of virions were observed: single‐ and double‐enveloped hexagonal virions. Based on size and morphology, these virions resemble iridovirus or herpesvirus. The cause of SDS is unknown, but the pathological changes, especially the vasculitis, suggest an infectious aetiology, possibly viral.