bluuebunny/crossref_metadata_2025 huggingface dataset:
Late onset neonatal anaemia due to maternal anti‐Ge: possible association with destruction of eythroid progenitors
Authors: Arndt P. A. , Garratty G. , Daniels G. , Green C. A. , Wilkes A. M. , Hunt P. , Do J. , Glenn S. , Peterson D.
Published: April 2005

summary.  There have been no reports of severe haemolytic disease of the newborn (HDN) due to Gerbich (Ge) antibodies. Two babies with HDN due to anti‐Ge3, both born to the same mother, are described. The anti‐Ge appeared in the first pregnancy and was not detectable in the first trimester, the babies' reticulocyte and bilirubin values were not greatly elevated (similar to HDN due to Kell antibodies), and the anaemia in both cases was either not apparent or not severe until 2 to 4 weeks after birth. Ge antigens are found on glycophorins (GPs) C and D; GPC, like Kell, has been shown to be expressed early on erythroid progenitor cells. The maternal anti‐Ge3 was shown to promote phagocytosis of Ge+ early erythroid progenitors by monocytes (similar to what has been reported with anti‐K and K+ progenitor cells). Thus, anti‐Ge3 may cause immune destruction of erythroid progenitors and possibly suppression of erythropoiesis (which would explain the reticulocyte and bilirubin values seen in both cases). Anti‐Ge3 appears to be capable of causing severe HDN. We suggest that babies born to mothers with anti‐Ge should have their haemoglobin concentrations monitored for signs of anaemia for several weeks after birth. Functional assays may prove useful.
An X‐ray microscopy perspective on the effect of glutaraldehyde fixation on cells
Authors: JEARANAIKOON S. , ABRAHAM‐PESKIR J. V.
Published: April 2005

X‐ray microscopy (XRM) is the only microscopy technique that can provide high‐resolution (30 nm) imaging of biological specimens without the need to fix, stain or section them. We aim to determine the effect, if any, of glutaraldehyde fixation on algae cells from the XRM perspective and thus provide beneficial information for both X‐ray and electron microscopists on artefacts induced by glutaraldehyde fixation. Three species of microalgae, Microcystis aeruginosa, Anabaena spiroides and Chlorella vulgaris, were used in this study. XRM images were obtained from unfixed and glutaraldehyde‐fixed cells and cell diameter and percentage X‐ray absorbency were measured. The mean diameter of cells from fixed preparations was smaller than from unfixed preparations; the mean diameter of M. aeruginosa cells was significantly reduced from 3.92 µm in unfixed cells to 3.43 µm in fixed cells (P < 0.05); in C. vulgaris the diameter of cells was also significantly reduced from 3.50 µm in unfixed to 2.98 µm in fixed samples (P < 0.05); whereas there was no significant reduction in the diameter of A. spiroides cells (4.04–3.90 µm). The protein crosslinking mechanism of glutaraldehyde probably generated free water molecules, which play an important role in radiation damage induced by X‐rays. This was seen as mass loss and cell shrinkage, which in the present study occurred more frequently in fixed cells than in unfixed cells. In addition, we demonstrated that the uptake of glutaraldehyde by cells makes all protein constituents in the cell organize into a closely packed configuration, thus causing a rise in the percentage of X‐ray absorbency. In fixed cells, this rise was approximately by a factor of two compared with unfixed samples in which protein constituents inside the cell are arranged in their native form.
A South Australian population‐based study of congenital talipes equinovarus
Authors: Byron‐Scott R. , Sharpe P. , Hasler C. , Cundy P. , Hirte C. , Chan A. , Scott H. , Baghurst P. , Haan E.
Published: April 2005

The aims of this study were to provide a population‐based prevalence for congenital talipes equinovarus (CTEV), to conduct an epidemiological investigation into the risk factors for CTEV and describe associated features. The study used a retrospective case–control design of CTEV notified to the South Australian Birth Defects Register between 1986 and 1996 inclusive, linking characteristics of mother and baby from the perinatal data collection.

The prevalence of isolated CTEV was 1.1/1000 total births (n = 231). Four factors were significantly associated with an increased risk of CTEV: maternal Aboriginal race (ORadj = 2.0; 95% CI 1.1, 3.6), male gender (ORadj = 2.4; 95% CI 1.8, 3.2), maternal anaemia (ORadj = 1.8; 95% CI 1.0, 2.9) and maternal hyperemesis (ORadj = 3.6; 95% CI 1.3, 9.8). The prevalence of CTEV associated with another birth defect or syndrome (n = 157) was 0.7/1000 total births. CTEV was associated with specific birth defects and also with oligohydramnios when another birth defect was present.
Risk factors for isolated talipes equinovarus in Western Australia, 1980–1994
Authors: Carey M. , Mylvaganam A. , Rouse I. , Bower C.
Published: April 2005

A case–control study based on routinely collected data for a West Australian (WA) cohort of births born 1980–94 inclusive was used to identify potential risk factors for the foot deformity isolated talipes equinovarus.

Race and sex were identified as risk factors and risk modifiers of the condition in WA infants. In comparison with their female counterparts, Aboriginal males were at greater risk of the deformity than Caucasian males (odd ratio [OR] 7.15, 95% confidence interval [CI] 2.68, 21.12 vs. OR 1.63, 95% CI 1.24, 2.15 respectively). Sex‐specific risk estimates of the deformity showed that Aboriginal males were at more than four times the risk of Caucasian males (OR 4.27, 95% CI 2.30, 8.25 respectively), but the risk amongst Aboriginal females was not dissimilar to the risk amongst Caucasian females (OR 1.12, 95% CI 0.49, 2.45).

Intrauterine constraint of the fetus was not found to be an important contributing factor to the deformity after accounting for the effect of all risk factors modelled in multivariable analyses. Indicators of intrauterine constraint including prolonged gestation, high infant birthweight, young maternal age (<20 years) and breech presentation were not associated with excess numbers of isolated talipes equinovarus cases.

Limited numbers of cases associated with other indicators of intrauterine constraint, including the specific categories of short maternal stature and a twin pregnancy prevented definitive conclusions regarding their association with the deformity. Improved data quality for gestational age estimates and oligohydramnios status are required before determining the prenatal risk impact of season of conception and of reduced amniotic fluid levels, respectively, on the occurrence of the deformity.
Consanguineous marriages in Jordan: why is the rate changing with time?
Authors: Hamamy H , Jamhawi L , Al‐Darawsheh J , Ajlouni K
Published: April 2005

The objective of this study was to explore the secular trend in consanguinity in Jordan and the subtypes of consanguineous marriages that may be undergoing a change. A total of 1032 individuals attending a diabetic clinic in Amman were interviewed. The questionnaire provided information on consanguinity status and date of marriage among three generations: the persons interviewed, their parents, parents of their spouses and their offspring. Data on consanguinity status among 5401 marriages was obtained. Generations were named generation 1 for marriages contracted before 1950, generation 2 for marriages contracted between 1950 and 1979, and generation 3 for marriages contracted after 1980. For generations 1, 2, and 3, first‐cousin marriages constituted 20.2, 28.5 and 19.5% of all marriages, respectively, while the subtype of paternal parallel first‐cousin marriages constituted 75.6, 60.3 and 43.3% of all first‐cousin marriages, respectively. The offspring of first‐cousin parents were significantly more prone to marry their relatives than the offspring of non‐consanguineous parents, with rates of first‐cousin marriages among offspring of first‐cousin parents and non‐related parents constituting 25.3 and 17.1% of all marriages, respectively. For generations 1, 2 and 3, the average coefficient of inbreeding was 0.0135, 0.02 and 0.0142, respectively. In conclusion, first‐cousin marriage rate among a representative population from Amman showed a significant decline among marriages contracted after 1980 compared to marriages contracted between 1950 and 1979, but not to marriages contracted before 1950. The proportion of paternal parallel first cousins among first‐cousin marriages showed a steady decline from one generation to the next.
X‐linked mental retardation: further lumping, splitting and emerging phenotypes
Authors: Kleefstra T , Hamel BCJ
Published: April 2005

X‐linked mental retardation (XLMR) is a very heterogeneous condition, subdivided in two categories mainly based on clinical features: syndromic XLMR (MRXS) and non‐syndromic XLMR (MRX). Although it was thought that 20–25% of mental retardation (MR) in males was caused by monogenetic X‐linked factors, recent estimations are lower: in the range of 10–12%. The number of identified genes involved in XLMR has been rapidly growing in the past years. Subsequently, an increasing number of patients and families have been reported in which mutations in XLMR genes have been identified. It was observed previously, that mutations in several of XLMR genes can result in syndromic and in non‐syndromic phenotypes. This observation has been confirmed for the more recently identified genes. Therefore, in this review, focus has been given on the clinical data and on phenotype‐genotype correlations for those genes implicated in both non‐syndromic and syndromic XLMR.
Communication about genetic testing in families of male BRCA1/2 carriers and non‐carriers: patterns, priorities and problems
Authors: Hallowell N , Ardern‐Jones A , Eeles R , Foster C , Lucassen A , Moynihan C , Watson M
Published: April 2005

This qualitative interview study explored the way in which information about predictive BRCA1/2 testing and its implications for children is disseminated within the families of at‐risk men who undergo genetic testing. Twenty‐nine in‐depth interviews were carried out with family members [male patients (n = 17), their partners (n = 8) and adult children (n = 4)]. These explored the following themes: experiences of cancer and genetic testing, decision‐making about testing and the communication of test results and genetic information within the immediate family. The interviews revealed that both male patients and their partners perceive themselves, rather than health professionals, as responsible for disclosing information about genetic testing and genetic risks to their children. Parents described three different communication strategies for the disclosure of genetic information to their children: complete openness, limited disclosure and total secrecy. The adoption of a particular communication strategy was justified in terms of children's rights to information vs their parental duties to protect their children from anxiety‐provoking information. Some of the problems arising from the adoption of different disclosure patterns are identified and the implications for clinical practice are discussed.
Demented flies? using Drosophila to model human neurodegenerative diseases
Authors: Michno K , Van De Hoef D , Wu H , Boulianne GL
Published: April 2005

The success of biomedical research in the past few decades has led to dramatic improvements in human health and, as a result, increased life expectancy. An unexpected consequence, however, has been an increase in the number of age‐related diseases and, in particular, neurodegenerative diseases. Despite their prevalence, a therapeutic void exists in part due to an incomplete understanding of the biochemical pathogenesis of these diseases. A powerful method that can be used to understand the basic mechanisms underlying neurodegenerative diseases is to generate animal models based on manipulating the expression of single genes that are disease causative. This approach has been facilitated by the fact that many neurodegenerative diseases are inherited as autosomal dominant traits such that expression of the mutant gene in a model organism might be expected to recapitulate the disease. During the past few years, the fruit fly, Drosophila melanogaster, has emerged as a powerful tool to model human neurodegenerative diseases. Here, we describe the various approaches utilized to create fly models of human neurodegenerative disease, and how they can aid in our understanding of disease pathogenesis and facilitate drug discovery and testing.
Distribution and redistribution of sulphur taken up from nutrient solution during vegetative growth in barley
Authors: Adiputra I. Gede K. , Anderson J. W.
Published: April 2005

Barley plants were grown in a nutrient solution containing 25 μM sulphate and the roots were pulsed with [35S]sulphate for 48‐h periods at 6 different times between the emergence of leaf 5 (L5) and the emergence of leaf 8 (L8). Growth was continued in unlabelled solution until the emergence of L10. Within the shoot system sulphur was directed principally into the leaf undergoing expansion. A large proportion of the 35S‐label delivered to young expanding leaves (> 40% of full expansion) did not occur at the time of the pulse, but subsequently during the ensuing chase indicating slow redistribution of sulphur from another site. During the later stages of leaf expansion (40–100%), most of the sulphur entered the leaf during the pulse, suggesting that sulphur was delivered more directly from the nutrient solution. Up to 75% of the sulphur delivered to L3–L6 at the time they approached or attained full expansion (70–100%) was re‐exported. At least some of the sulphur exported from fully expanded leaves was redistributed to developing leaves.
Carbohydrate status in dormant and afterripened excised wild oat embryos
Authors: Foley Michael E. , Bancal Marie‐Odile , Nichols Maxine B.
Published: April 2005

Germination and carbohydrate concentrations were determined in excised dormant and afterripened wild oat (Avena fatua L. line M73) embryos cultured on N6 medium with and without 88 mM fructose (Fru). Without Fru dormant embryos began to germinate after approximately 2 weeks, and the germination rate was greater at 12 than 16°C. With addition of Fru 80% of dormant embryos germinated in 3 days. More than 80% of afterripened embryos germinated within 1 day on N6 with or without additional sugars. Therefore, relative to afterripened embryos, true embryo dormancy exists in line M73. Concentrations of starch and soluble sugars were initially similar in dormant and afterripened embryos. Culturing dormant and afterripened embryos on medium with Fru resulted in concentrations of glucose (Glu), sucrose (Sue), Fru and maltose (Mal) that were the same or higher than the initial levels. The concentration of starch in embryos initially increased slightly then remained constant or declined, except in dormant embryos on Fru‐amended medium, where starch accumulated to 34 μg Glu equivalents (mg fresh weight)‐1 at 52 h. Raffinose (Raf) and stachyose (Stach) concentrations declined over time in all embryos. Carbohydrate concentrations in afterripened embryos on medium without Fru decreased to nearly undetectable levels by 52 h. Soluble sugar concentrations in dormant embryos on medium without Fru also declined by 52 h, but changes were not as extensive as those in afterripened embryos without Fru. In 52 h Raf and Stach were nearly depleted in all afterripened embryos, and in dormant embryos cultured on Fru‐containing medium but not in dormant embryos without Fru. The concentration of Stach in dormant embryos without Fru declined 60% at 12 to 18 days coinciding with the potential for germination. The results demonstrate that a decline in Stach concentration is associated with the potential for germination of dormant (D) excised embryos. The mechanism of dormancy‐breaking associated with the Raf family oligosaccharides remains to be determined.
The effects of excess manganese on photosynthetic rate and concentration of chlorophyll in Triticum aestivum grown in solution culture
Authors: Macfie Sheita M. , Taylor Gregory J.
Published: April 2005

Manganese toxicity, which involves a broad array of physiological responses, has been identified as an important factor limiting plant growth on acid soils. In the experiments reported here, we examined the toxic effects of Mn on chlorophyll content, photosynthesis and respiration in two cultivars (Norquay and Columbus) of Triticum aestivum (wheat) which differ in tolerance of Mn. When grown over a range of concentrations of Mn (0–1 000 μM), the Mn‐tolerant cultivar maintained higher rates of photosynthesis and respiration, and higher concentrations of chlorophyll a and chlorophyll b, than did the Mn‐sensitive cultivar, despite greater accumulations of Mn in leaf tissues. After 5 days growth with 1 000 μM Mn in solution, the photosynthetic rate fell to 25% of control in the sensitive cultivar and to only 75% of control in the tolerant cultivar. The concentration of chlorophyll a fell to 50% of control in the sensitive cultivar, but did not differ from control in the tolerant cultivar. Greater effects were seen on concentrations of chlorophyll b. which fell to 35% and 55% of control in the sensitive and tolerant cultivars, respectively. Rates of photosynthesis decreased in both cultivars as concentrations of chlorophyll decreased; however, the photosynthetic rate per unit chlorophyll remained constant or increased in the tolerant cultivar and decreased in the sensitive cultivar as concentrations of Mn in solution increased. Thus, in the sensitive cv. Columbus, Mn seemed to have a toxic effect on both chlorophyll content and photosynthesis per unit chlorophyll. In the tolerant cv. Norquay, the only clear effect of Mn was a reduction in chlorophyll content, although direct inhibition of photosynthesis could not be discounted.
A survey of perceptions and attitudes among European surgeons towards the clinical impact and management of postoperative ileus
Authors: Kehlet H. , Williamson R. , Büchler M. W. , Beart R. W.
Published: April 2005

Objective  Postoperative ileus (POI) can negatively affect patient recovery and morbidity, yet the lack of an internationally accepted definition and clinical management pathway for this condition suggest POI may be under‐recognized as a clinical problem. The purpose of this survey was therefore to assess current attitudes of surgeons towards the clinical impact and management of POI.

Subjects and methods  Telephone interviews were conducted with 230 surgeons from hospitals in the UK, France, Germany, Italy and Spain.

Results  Across Europe, there are differences in the terms surgeons use to refer to delayed recovery of gastrointestinal (GI) function and the symptoms, concerns and risks they associate with this condition. Furthermore, there is marked variation in the attitudes of European surgeons towards minimizing the risk of delayed recovery of GI function and in the strategies to manage POI. Additionally, some of the measures applied most commonly by European surgeons are in contrast to evidence in the literature indicating that they have no benefit for quicker resolution of GI function.

Conclusion  The results suggest that there is a need for clearer definition of the factors that constitute POI, increased recognition of the impact of this condition and improved understanding of the most effective peri‐/postoperative care for surgical patients.
The role of prophylactic oophorectomy in women undergoing surgery for colorectal cancer
Authors: Banerjee S. , Kapur S. , Moran B. J.
Published: April 2005

Background  Current gynaecological practice is to remove surgically accessible ovaries with prior consent in post menopausal women to eradicate the risk of subsequent development of ovarian cancer. However in colorectal surgery opinion is divided, although evidence suggests that ovarian metastases from colorectal adenocarcinoma occur in 3–4% of cases.

Methods  The review is based on published literature of the role of prophylactic oophrectomy in women undergoing surgery for colorectal cancer, obtained from Medline/PubMed and other online databases using the terms ‘prophylactic oophorectomy’ and ‘colon cancer’. Additional references were sourced by manually searching bibliographies of articles located.

Results and conclusion  There is a paucity of published information and published reports have conflicting conclusions. A preliminary report of a randomised prospective trial suggests the probability of increased recurrence free survival in the oophorectomy group. It would seem appropriate to consider the necessity and possible benefits of oophorectomy in women prior to surgery for colorectal cancer. This would seem particularly relevant in distal sigmoid or upper rectal cancers when the ovaries or uterus may be directly invaded and curative surgery would involve en bloc resection. Post menopausal women with a family history of ovarian cancer should be advised to have oophorectomy.
Impact of faecal incontinence severity on health domains
Authors: Deutekom M. , Terra M. P. , Dobben A.C. , Dijkgraaf M. G. W. , Baeten C. G. M. I. , Stoker J. , Bossuyt P. M. M.
Published: April 2005

Objective  Faecal incontinence is a problem that can have a major impact on the quality of life of those affected. Our aim was to relate the severity of faecal incontinence to the impact on several general health domains.

Methods  Patients from a prospective diagnostic cohort study, performed in 16 medical centres in the Netherlands, were invited to the study. The severity of incontinence was determined with the Vaizey score, which ranges from 0 (continent) to 24 (totally incontinent). Based on their Vaizey score, patients were assigned to one of five severity categories. All patients completed the EuroQol‐5D instrument, which evaluates the existence of problems on five health domains: mobility, self‐care, usual activities, pain/discomfort and anxiety/depression.

Results  Data from 259 consecutive patients (25 male) could be analysed. Their mean age was 59 years (SD ± 12). The mean duration of faecal incontinence was 8.1 years (SD ± 8). The proportion of patients reporting problems rose significantly with increasing severity of faecal incontinence in the domains of usual activities (ranging from 36% in the least severe group to 71% in the most severe group (P < 0.001)), pain/discomfort (ranging from 35% to 60%; P = 0.025), and anxiety/depression (ranging from 23% to 49%; P = 0.037). No significant trends could be observed in the domains of mobility and self‐care.

Conclusion  There exists a significant relation between severity of incontinence and frequency of reported problems in the domains of usual activities, pain/discomfort and anxiety/depression.
Long‐term outcomes of restorative proctocolectomy for Crohn's disease and indeterminate colitis
Authors: Tekkis P. P. , Heriot A. G. , Smith O. , Smith J. J. , Windsor A. C. J. , Nicholls R. J.
Published: April 2005

Introduction  The present study aims to evaluate the short‐term and long‐term outcomes of patients undergoing restorative proctocolectomy (RPC) for Crohn's disease (CD) and Indeterminate colitis (IC) and to identify factors associated with adverse outcomes.

Methods  A descriptive study of 52 patients with CD or IC from a total of 1652 patients undergoing primary or salvage RPC in a single tertiary referral centre between 1978 and 2003. Primary outcomes were ileal pouch failure (excision or indefinite diversion), adverse events and functional outcomes (bowel frequency, urgency and continence).

Results  Patients with IC or IC favouring ulcerative colitis (Group 1, n = 26) had a pouch failure rate of 11.5%vs 57.5% for patients with CD or IC favouring CD (Group 2, n = 26). Pouch salvage surgery was undertaken in 15 patients with a 13.3% failure rate. Patients in Group 2 were 2.6 times more likely (95% CI: 0.96–7. No significant differences were evident between CD and IC patients with regards to pelvic sepsis (19.2%vs 15.4%), anastomotic stricture (23.1%vs 21.7%), small bowel obstruction (26.9%vs 26.9%) or pouchitis (15.4%vs 11.5%). The 24‐h bowel frequency (7.5 vs 8), faecal urgency, daytime or night time incontinence were similar between patients with CD or IC..17) to develop a pouch‐related fistula than patients in Group 1.

Discussion  Crohn's disease and to a great extent indeterminate colitis favouring CD were both associated with high failure rates and postoperative pouch‐related fistula rates. Despite these problems, functional outcomes for patients with CD or IC were similar. Patients with IC should remain candidates for RPC but careful pre‐operative assessment is advised to exclude clinical signs favouring the diagnosis of CD. The complications associated with failure are extensive and the option of reconstructive surgery in patients with CD should be questioned.
Ileostomy for constipation: long‐term postoperative outcome
Authors: Scarpa M. , Barollo M. , Keighley M. R. B.
Published: April 2005

Background and aims  Idiopathic constipation is a rare indication for ileostomy construction. The aim of the study was to evaluate the success of ileostomy in treatment of severe constipation. Also to analyse the surgical complications and re‐operation rate to identify any factors potentially predictive of outcome.

Patients and methods  This retrospective study analysed the long‐term outcome of 24 ileostomies constructed for constipation. The ileostomy construction was performed in 13 patients during large bowel/rectum resection, in 6 after a full laparotomy and in 5 through an abdominal wall trephine alone. We analysed the surgical complications and the re‐operation rate according any factors potentially predictive of outcome.

Results  One (4%) patient had persistent constipation after stoma creation. Surgical complications occurred in 11 (46%): retraction in 6 (25.0%), peristomal sepsis in 3 (12.5%) and parastomal hernia in 2 (8.1%). Refashioning of the stoma was necessary in 7 (29%) patients. Previous abdominal surgery, end ileostomy, ileostomy constructed after large bowel resection or laparotomy were associated with a significantly higher incidence of stomal complications while age, duration of follow up, major complication and ileostomy created after bowel resection were associated to a significantly higher re‐operation rate (P < 0.05). Multivariate analysis identified end ileostomy and ileostomy created after bowel resection as independent risk factors for surgical complication and re‐operation, respectively (P < 0.05).

Conclusions  Ileostomies were associated with a high frequency of complications, but most could be managed by minor surgical interventions. Patients who are considered for an ileostomy for severe idiopathic constipation should, where possible, have a loop ileostomy through a trephine rather than a laparotomy.
Autopsy your dead…and living: a proposal for fisheries science, fisheries management and fisheries
Authors: Smith Tim D. , Link Jason S.
Published: April 2005

Ghoti papers Ghoti aims to serve as a forum for stimulating and pertinent ideas. Ghoti publishes succinct commentary and opinion that addresses important areas in fish and fisheries science. Ghoti contributions will be innovative and have a perspective that may lead to fresh and productive insight of concepts, issues and research agendas. All Ghoti contributions will be selected by the editors and peer reviewed.

Etymology of Ghoti George Bernard Shaw (1856‐1950), polymath, playwright, Nobel prize winner, and the most prolific letter writer in history, was an advocate of English spelling reform. He was reportedly fond of pointing out its absurdities by proving that ‘fish’ could be spelt ‘ghoti’. That is: ‘gh’ as in ‘rough’, ‘o’ as in ‘women’ and ‘ti’ as in palatial.

Concerns abound about the state of the world's fisheries, and by association fisheries science and fisheries management. An important question is the cause of the problems with fisheries, which are managed based on advice given by practioners of the discipline of fisheries science. These scientists provide advice in two distinct forms, one ‘policy supporting’ and the other ‘policy forming’. Policy supporting advice is given within an existing management setting and is typically provided only after rigorous and standardized evaluation. In contrast, there is no formal basis for evaluating policy‐forming advice. Criteria for evaluating the adequacy of policy forming advice are difficult to define, and the absence of such criteria poses difficulties for both fisheries scientists and managers. Following a medical practice analogy, we propose rigorously conducting ‘autopsies’ of fisheries as an approach for evaluating policy‐forming advice. A systematic approach to conducting such autopsies is suggested, with the recognition that this may be crucial for the further development of fisheries science as a scientific discipline rather than as an art form.
A comprehensive study of the use of a homologous promoter in antisense cotton lines exhibiting a high seed oleic acid phenotype
Authors: Sunilkumar Ganesan , Campbell LeAnne M. , Hossen Monjur , Connell James P. , Hernandez Ernesto , Reddy Avutu S. , Smith Carl W. , Rathore Keerti S.
Published: April 2005

As opposed to first‐generation biotechnology products, such as pest‐resistant crops and herbicide‐resistant crops, second‐generation products often utilize plant‐derived, homologous or heterologous genes and/or promoters. In this study, we evaluated the ability of a promoter from a gene encoding a major storage protein in cottonseed to drive an antisense sequence of the cotton FAD2 gene to down‐regulate the activity of Δ‐12 desaturase enzyme in cottonseeds. The oleic acid level in the transgenic cottonseeds approximately doubled from the wild‐type level of 15%, with a concomitant decrease in the level of linoleic acid. A more extensive study of one line revealed a higher degree of seed‐to‐seed variability in the transgenic phenotype. A thorough investigation was conducted to determine the impact of the use of a homologous promoter to drive a transgene on the activity of the endogenous promoter. The results showed that the use of the homologous α‐globulin B promoter for transgenic purposes did not adversely affect the expression of α‐globulin B storage protein in cottonseed. The results obtained in this investigation on the use of a homologous promoter and antisense technology will be useful in the design of strategies to alter biosynthetic pathways for nutritional quality improvements and for the production of heterologous proteins of commercial value in seeds.
Mutan produced in potato amyloplasts adheres to starch granules
Authors: Kok‐Jacon Géraldine A. , Vincken Jean‐Paul , Suurs Luc C. J. M. , Visser Richard G. F.
Published: April 2005

Production of water‐insoluble mutan polymers in Kardal potato tubers was investigated after expression of a full‐length (GtfI) and a truncated mutansucrase gene referred to as GtfICAT (GtfI without glucan‐binding domain) from Streptococcus downei. Subsequent effects on starch biosynthesis at the molecular and biochemical levels were studied. Expression of the GtfICAT gene resulted in the adhesion of mutan material on starch granules, which stained red with erythrosine, and which was hydrolysed by exo‐mutanase. In addition, GtfICAT‐expressing plants exhibited a severely altered tuber phenotype and starch granule morphology in comparison to those expressing the full‐length GtfI gene. In spite of that, no structural changes at the starch level were observed. Expression levels of the sucrose‐regulated, AGPase and GBSSI genes were down‐regulated in only the GTFICAT transformants, showing that GtfICAT expression interfered with the starch biosynthetic pathway. In accordance with the down‐regulated AGPase gene, a lower starch content was observed in the GTFICAT transformants. Finally, the rheological properties of the GTFICAT starches were modified; they showed a higher retrogradation during cooling of the starch paste.
Adult‐onset Still's disease with atypical cutaneous features
Authors: Affleck AG , Littlewood SM
Published: April 2005

Introduction  The diagnosis of adult‐onset Still's disease (AOSD) can be very difficult. There are no specific tests and reliance is usually placed on a symptom complex and the well described typical rash seen in most patients. In recent years, however, other cutaneous manifestations of AOSD have been reported but these are not so well known.

Observations  We report a patient with urticaria and fixed plaques and review the other ‘atypical’ cutaneous findings associated with AOSD.

Conclusions  The diagnosis of AOSD can be made in the absence of the typical Still's rash but in the presence of other atypical cutaneous features.
Simultaneous occurrence of pure red cell aplasia and papular‐purpuric ‘gloves and socks’ syndrome in parvovirus B‐19 infection
Authors: Loukeris D , Serelis J , Aroni K , Tsakris A , Voulgarelis M
Published: April 2005

Papular‐purpuric ‘gloves and socks’ syndrome (PPGSS) has been associated with parvovirus B‐19 infection. We report a case of an adult immunocompetent male who presented with PPGSS. Bone marrow examination revealed pure red cell aplasia. Parvovirus B‐19 DNA was detected by polymerase chain reaction in the patient's serum, whole blood and in the cutaneous lesions. This report illustrates the variety of clinical manifestations caused by B‐19 infection, presents for the first time the concurrent appearance of pure red cell aplasia and PPGSS in the same patient and, finally, suggests that PPGSS may be due to direct lytic effect of the virus.
Dermatological manifestations in hepatitis B surface antigen carriers in east region of Turkey
Authors: Dogan B
Published: April 2005

Background and design   Various cutaneous disorders associated with hepatitis B virus infection have been reported so far. Almost all of them were some peculiar eruptions or some disorders mainly based on immune complex reaction.

Objective  The aim of this study was to compare the frequency of all dermatological disorders other than genetic ones in hepatitis B surface antigen (HBsAg) carrier patients and controls in our geographical area.

Methods  Examinations were made on 88 hepatitis B carriers and 84 control patients, including entire skin surface, and oral and anogenital mucosa, and all the manifestations that could be seen were noted down.

Results  While the number of HBsAg carrier patients with dermatological disorders was 62 (70.75%), it was only 24 (28.57%) in control patients. This seems statistically important: Yates’ corrected chi‐square test = 28.5; P = 0.0000001; odds ratio = 6.0 (2.94 < odds ratio < 12.2). The prevalence of oral lichen planus and pitted keratolysis in HBsAg carrier group was significantly higher than that of controls. We found the duration of positivity was not important statistically in the development of the dermatological disorders.

Conclusions  HBsAg positivity may induce or cause proneness to oral lichen planus and pitted keratolysis with some mechanism that needs to be elucidated.
Juvenile colloid milium: a case report
Authors: Ekmekci TR , Koslu A , Sakiz D
Published: April 2005

Juvenile colloid milium is extremely rare, has its onset before puberty, may be inherited, may be caused by ultraviolet light, and is thought to result from the degeneration of keratinocytes. We report a 7‐year‐old girl with juvenile colloid milium. To the best of our knowledge this is the first reported case in Turkey.
Endoplasmic reticulum positioning and partitioning in mitotic HeLa cells
Authors: McCullough Simon , Lucocq John
Published: April 2005

Cell division serves to distribute chromosomes and organelles into two daughter cells, but the mechanism of rough endoplasmic reticulum (RER) segregation in animal cell mitosis is poorly understood. Here we study the distribution of RER in mitotic HeLa cells and its relation to the cytoskeleton. At metaphase, the RER was located in the cell cortex and was most concentrated in two locations. Close to the plasma membrane the RER was closely associated with cortical actin, and after treatment with Latrunculin A RER elements retracted to the deep cortex and became more tubular. Positioning was therefore dependent on cortical F‐actin. Deeper in the cortex cisternae were wrapped tightly around the contours of the spindle body and orientated along microtubules close the spindle poles. Stereology revealed a close correlation between RER volume and cell volume in telophase daughter cells. These results suggest that the RER is positioned at the outer and inner regions of metaphase cortex by association with cytoskeleton. This arrangement combined with a disposition in concentric layers, deep to the plasma membrane, appears to distribute the RER evenly in the cortex and may help to couple quantities of RER and cell constituents.
Dopamine D2 receptor stimulation of mitogen‐activated protein kinases mediated by cell type‐dependent transactivation of receptor tyrosine kinases
Authors: Wang Chunhe , Buck David C. , Yang Rui , Macey Tara A. , Neve Kim A.
Published: April 2005

Dopamine D2 receptor activation of extracellular signal‐regulated kinases (ERKs) in non‐neuronal human embryonic kidney 293 cells was dependent on transactivation of the platelet‐derived growth factor (PDGF) receptor, as demonstrated by the effect of the PDGF receptor inhibitors tyrphostin A9 and AG 370 on quinpirole‐induced phosphorylation of ERKs and by quinpirole‐induced tyrosine phosphorylation of the PDGF receptor. In contrast, ectopically expressed D2 receptor or endogenous D2‐like receptor activation of ERKs in NS20Y neuroblastoma cells, which express little or no PDGF receptor, or in rat neostriatal neurons was largely dependent on transactivation of the epidermal growth factor (EGF) receptor, as demonstrated using the EGF receptor inhibitor AG 1478 and by quinpirole‐induced phosphorylation of the EGF receptor. The D2 receptor agonist quinpirole enhanced the coprecipitation of D2 and EGF receptors in NS20Y cells, suggesting that D2 receptor activation induced the formation of a macromolecular signaling complex that includes both receptors. Transactivation of the EGF receptor also involved the activity of a matrix metalloproteinase. Thus, although D2 receptor stimulation of ERKs in both cell lines was decreased by inhibitors of ERK kinase, Src‐family protein tyrosine kinases, and serine/threonine protein kinases, D2‐like receptors activated ERKs via transactivation of the EGF receptor in NS20Y neuroblastoma cells and rat embryonic neostriatal neurons, but via transactivation of the PDGF receptor in 293 cells.
Integrin signaling cascades are operational in adult hippocampal synapses and modulate NMDA receptor physiology
Authors: Bernard‐Trifilo Joie A. , Kramár Enikö A. , Torp Reidun , Lin Ching‐Yi , Pineda Eduardo A. , Lynch Gary , Gall Christine M.
Published: April 2005

Integrin class adhesion proteins are concentrated at adult brain synapses. Whether synaptic integrins engage kinase signaling cascades has not been determined, but is a question of importance to ideas about integrin involvement in functional synaptic plasticity. Accordingly, synaptoneurosomes from adult rat brain were used to test if matrix ligands activate integrin‐associated tyrosine kinases, and if integrin signaling targets include NMDA‐class glutamate neurotransmitter receptors. The integrin ligand peptide Gly‐Arg‐Gly‐Asp‐Ser‐Pro (GRGDSP) induced rapid (within 5 min) and robust increases in tyrosine phosphorylation of focal adhesion kinase, proline‐rich tyrosine kinase 2 and Src family kinases. Increases were similarly induced by the native ligand fibronectin, blocked with neutralizing antibodies to β1 integrin, and not obtained with control peptides, indicating that kinase activation was integrin‐mediated. Both GRGDSP and fibronectin caused rapid Src kinase‐dependent increases in tyrosine phosphorylation of NMDA receptor subunits NR2A and NR2B in synaptoneurosomes and acute hippocampal slices. Tests of the physiological significance of the latter result showed that ligand treatment caused a rapid and β1 integrin‐dependent increase in NMDA receptor‐mediated synaptic responses. These results provide the first evidence that, in adult brain, synaptic integrins activate local kinase cascades with potent effects on the operation of nearby neurotransmitter receptors implicated in synaptic plasticity.
Regulation of mitochondrial gene expression by energy demand in neural cells
Authors: Mehrabian Zara , Liu Li‐Ing , Fiskum Gary , Rapoport Stanley I. , Chandrasekaran Krish
Published: April 2005

Mitochondrial DNA (mtDNA) encodes critical subunit proteins of the oxidative phosphorylation (OXPHOS) complex that generates ATP. This study tested the hypothesis that mitochondrial gene expression in neural cells is regulated by energy demand, as modified via stimulation of cellular sodium transport. Exposure of PC12S cells to the sodium ionophore monensin (250 nm) for 1–6 h caused a 13–60% decrease in cellular ATP (from 15 to 5 nmol per mg protein at 6 h). Levels of mitochondrial DNA‐encoded mRNAs (mt‐mRNAs) increased significantly (150%) within the first hour of exposure to monensin, and then decreased significantly (50%) at 3–4 h. Levels of mtDNA‐encoded 12S rRNA and nuclear DNA‐encoded OXPHOS subunit mRNAs were not significantly affected. Exposure of primary cerebellar neuronal cultures to the excitatory amino acid glutamate caused a similar rapid and significant increase followed by a significant decrease in cell mt‐mRNA levels. The monensin‐induced initial increase in mt‐mRNA levels was abolished by pretreatment with actinomycin D or by reducing extracellular sodium ion concentration. The monensin‐induced delayed reduction in mt‐mRNA levels was accelerated in the presence of actinomycin D, and was accompanied by a 67% reduction in the half‐life (from 3.6 to 1.2 h). Exposure of PC12S cells to 2‐deoxy‐d‐glucose significantly decreased cellular ATP levels (from 14.2 to 7.1 nmol per mg protein at 8 h), and increased mt‐mRNA levels. These results suggest a physiological transcriptional mechanism of regulation of mitochondrial gene expression by energy demand and a post‐transcriptional regulation that is independent of energy status of the cell.
Influences of dopaminergic lesion on epidermal growth factor‐ErbB signals in Parkinson's disease and its model: neurotrophic implication in nigrostriatal neurons
Authors: Iwakura Yuriko , Piao Ying‐shan , Mizuno Makoto , Takei Nobuyuki , Kakita Akiyoshi , Takahashi Hitoshi , Nawa Hiroyuki
Published: April 2005

Epidermal growth factor (EGF) is a member of a structurally related family containing heparin‐binding EGF‐like growth factor (HB‐EGF) and transforming growth factor alpha (TGFα) that exerts neurotrophic activity on midbrain dopaminergic neurons. To examine neurotrophic abnormality in Parkinson's disease (PD), we measured the protein content of EGF, TGFα, and HB‐EGF in post‐mortem brains of patients with Parkinson's disease and age‐matched control subjects. Protein levels of EGF and tyrosine hydroxylase were decreased in the prefrontal cortex and the striatum of patients. In contrast, HB‐EGF and TGFα levels were not significantly altered in either region. The expression of EGF receptors (ErbB1 and ErbB2, but not ErbB3 or ErbB4) was down‐regulated significantly in the same forebrain regions. The same phenomenon was mimicked in rats by dopaminergic lesions induced by nigral 6‐hydroxydopamine infusion. EGF and ErbB1 levels in the striatum of the PD model were markedly reduced on the lesioned side, compared with the control hemisphere. Subchronic supplement of EGF in the striatum of the PD model locally prevented the dopaminergic neurodegeration as measured by tyrosine hydroxylase immunoreactivity. These findings suggest that the neurotrophic activity of EGF is maintained by afferent signals of midbrain dopaminergic neurons and is impaired in patients with Parkinson's disease.
Real‐time quantitative PCR analysis of temporal–spatial alterations in gene expression after spinal cord contusion
Authors: Wu Xiaofang , Yoo Soonmoon , Wrathall Jean. R.
Published: April 2005

Rat spinal cord contusion injury models the histopathology associated with much clinical spinal cord injury (SCI). Studies on altered gene expression after SCI in these models may identify therapeutic targets for reducing secondary injury after the initial trauma and/or enhancing recovery processes. However, complex spatial and temporal alterations after injury could complicate interpretation of changes in gene expression. To test this hypothesis, we selected six genes and studied their temporal and spatial patterns of expression at 1 h, 1, 3 and 7 days after a standardized spinal cord contusion produced by a weight drop device (10 g × 25 mm at T8). Real‐time RT–PCR using TaqMan probes was employed to quantify mRNA for proteolipid protein, glyceraldehyde‐3‐phosphate dehydrogenase, glial fibrillary acidic protein, nestin, and the GluR2 and NR1 subunits of glutamate receptors. We found widely different temporal and spatial patterns of altered gene expression after SCI, including instances of opposing up‐ and down‐regulation at different locations in tissue immediately adjacent to the injury site. We conclude that greater use of the reliable and extremely sensitive technique of quantitative real‐time PCR for regional tissue analysis is important for understanding the altered gene expression that occurs after CNS trauma.
Vaccination alone or in combination with pyridostigmine promotes and prolongs activation of stress‐activated kinases induced by stress in the mouse brain
Authors: Wang Dechun , Perides George , Liu Ya Fang
Published: April 2005

Gulf war illnesses (GWI) are currently affecting thousands of veterans. To date, the molecular mechanisms underlying the pathogenesis of these illnesses remain unknown. During Gulf war I, military personnel were exposed to multiple stressors, one or more vaccines, pyridostigmine (PY), and other chemicals. In our previous studies, we found that stress induces activation of mitogen activated protein‐kinase kinase 4 (MKK4) and c‐Jun‐N‐terminal kinase (JNK) in the mouse brain (Liu et al. 2004). Our working hypothesis is that stress, vaccination, and PY may synergistically induce activation of MKK4 and JNK in the brain, leading to over‐activation of these kinases and neurological injuries. To test our hypothesis, we examined the effect of keyhole limpet hemocyanin (KLH) immunization alone or in combination with PY on activation of MKK4 and JNK induced by stress. We found that KLH immunization alone had a small effect on MKK4 or JNK activity but it significantly enhanced and prolonged activation of these kinases induced by stress, from a few hours to several days. Additionally, KLH immunization caused activation of p38MAPK. PY treatment further enhanced and prolonged activation of these kinases induced by stress in combination with KLH immunization and triggered activation of caspase‐3. Our current studies suggest that stress, vaccination, and PY may synergistically act on multiple stress‐activated kinases in the brain to cause neurological impairments in GWI.
Mutations in the neurofilament light gene linked to Charcot‐Marie‐Tooth disease cause defects in transport
Authors: Pérez‐Ollé Raül , López‐Toledano Miguel A. , Goryunov Dmitry , Cabrera‐Poch Noemi , Stefanis Leonidas , Brown Kristy , Liem Ronald K. H.
Published: April 2005

Neurofilament light gene mutations have been linked to a subset of patients with Charcot‐Marie‐Tooth disease, the most common inherited motor and sensory neuropathy. We have previously shown that Charcot‐Marie‐Tooth‐linked mutant neurofilament light assembles abnormally in non‐neuronal cells. In this study, we have characterized the effects of expression of mutant neurofilament light proteins on axonal transport in a neuronal cell culture model. We demonstrated that the Charcot‐Marie‐Tooth‐linked neurofilament light mutations: (i) affect the axonal transport of mutant neurofilaments; (ii) have a dominant‐negative effect on the transport of wild‐type neurofilaments; (iii) affect the transport of mitochondria and the anterograde axonal transport marker human amyloid precursor protein; (iv) result in alterations of retrograde axonal transport and (v) cause fragmentation of the Golgi apparatus. Increased neuritic degeneration was observed in neuronal cells overexpressing neurofilament light mutants. Our results suggest that these generalized axonal transport defects could be responsible for the neuropathy in Charcot‐Marie‐Tooth disease.