bluuebunny/crossref_metadata_2025_split huggingface dataset:
JONATHAN EDWARDS IN HIS TIME, AND IN OURS
Authors: HALL DAVID D. 
Published: October 2004

George M. Marsden, Jonathan Edwards: A Life (New Haven, CT: Yale University Press, 2003)Robert E. Brown, Jonathan Edwards and the Bible (Bloomington: Indiana University Press, 2002)Avihu Zakai, Jonathan Edwards's Philosophy of History: The Re-enchantment of the World in the Age of Enlightenment (Princeton, NJ: Princeton University Press, 2003)Amy Plantinga Pauw, “The Supreme Harmony of All”: The Trinitarian Theology of Jonathan Edwards (Grand Rapids, MI: Eerdmans, 2002)We play tricks on the past, but the past also plays tricks on us. We try to fool the past by reconstructing it in our own image, imposing order and significance on the untidy sources we depend upon. The trick the past plays on us is to remain defiantly strange, ever able to expose what it is that our gestures of sympathetic reconstruction have altered, ignored, or suppressed.
RECONSTRUCTING GERMAN IDEALISM AND ROMANTICISM: HISTORICISM AND PRESENTISM
Authors: ZAMMITO JOHN 
Published: October 2004

Frederick Beiser, German Idealism: The Struggle Against Subjectivism, 1781–1801 (Cambridge, MA/London: Harvard University Press, 2002)Robert Richards, The Romantic Conception of Life: Science and Philosophy in the Age of Goethe (Chicago/London: University of Chicago Press, 2002)All art should become science and all science art; poetry and philosophy should be made one.Friedrich Schlegel, Kritische FragmenteWhen two major studies on the same thematic appear roughly simultaneously, integrating not only their authors' respective careers but the revisions of a whole generation of scholarship, the moment cries out for stock-taking, both substantively and methodologically. At a minimum, we need to recognize the key theses of our two protagonists and the frameworks they erect to uphold them. But we need even more to step back from that endeavor to wider considerations. I advance two claims in that light. First, something has been unearthed in these studies which speaks to urgent philosophical concerns of our day, namely the rise of naturalized epistemology and the need for a more encompassing naturalism. Indeed, I suspect this current interest may have incited (if only subliminally) discernment of just those aspects of the earlier age. That signals something essential about the point and practice of intellectual history, namely (my second claim) the mutuality, not opposition, of historicism and presentism.
Maternal Phenylketonuria Collaborative Study (MPKUCS)—The 'outliers'
Authors: Hanley W. B. , Azen C. , Koch R. , Michals‐Matalon K. , Matalon R. , Rouse B. , Trefz F. , Waisbren S. , de la Cruz F. 
Published: October 2004

AbstractSummary: Analysis of outcome data from 305 of the 414 offspring from the Maternal Phenylketonuria Collaborative Study (MPKUCS), plus 70 control offspring, revealed significant deficits in the IQ (intelligence quotient), as measured by the Wechsler Intelligence Scale for Children ‐ Revised (WISC‐R), when maternal metabolic control during pregnancy was delayed and/or inadequate. There were, however, 23 'outliers' (7.5% of the 305) in which the offspring's intellectual IQ was worse (n=10) or better (n=13) than expected. The aim of this study was to determine whether collection parameters were incomplete or whether these subjects were true biological variants influenced by other undetected factors or, perhaps, by modifier genes. Among the 10 offspring whose intellectual functioning was worse than expected, additional complications were uncovered that could explain the poor outcome. Four of the 13 offspring with higher than expected IQ had mothers with mild variants of PKU in which the insult to the fetus would not be expected to be as profound. For the other nine offspring whose intellectual performance was better than expected, there was no explanation, based on the parameters studied. We hypothesize that modifier genes will, at times, protect the fetus despite high maternal concentrations of phenylalanine. Not all offspring from the same (untreated) PKU mother may be similarly affected. Finding the source of these modifiers might effect the treatment of MPKU.
Development of pathogenic concepts in glutaryl‐CoA dehydrogenase deficiency: The challenge
Authors: Goodman S. I. 
Published: October 2004

AbstractSummary: The purpose of this review is to set the stage for discussions that follow about the biochemical and molecular bases of glutaric acidaemia type I, and about the pathogenesis of the characteristic acute striatal necrosis that often occurs during the first years of life.
Preliminary attempts to establish a rat model of striatal injury in glutaric acidaemia type I
Authors: Funk C. B. R. , Prasad A. N. , Bigio M. R. Del 
Published: October 2004

AbstractSummary: Glutaric acidaemia type I (GA I) is caused by the deficiency of glutaryl‐CoA dehydrogenase, resulting in accumulation of glutaric acid (GA) and 3‐ hydroxyglutaric acid (3‐OH‐GA) in blood and cerebrospinal fluid (CSF). Neuropathological changes with onset in childhood consist of severe neuronal loss in the caudate and putamen. An animal model is necessary to test possible intervention strategies, and prior reports suggested that GA or 3‐OH‐GA could be used to create specific neuron loss in adult rats. Adult, 3‐week‐old and 2‐week‐old rats received intrastriatal injections of GA and 3‐OH‐GA at a range of doses. High concentrations caused necrotic lesions in striatum. Low concentrations caused white‐matter axonal damage and small areas of neuron loss. Injection of lipopolysaccharide prior to administration of 3‐OH‐GA was not associated with enhanced neuronal loss. Our findings contradict prior claims and we conclude that the simple model of a single GA or 3‐OH‐GA injection into rat brain does not replicate the neuropathological findings in humans.
Modulation of glutamatergic and GABAergic neurotransmission in glutaryl‐CoA dehydrogenase deficiency
Authors: Wajner M. , KÖlker S. , Souza D. O. , Hoffmann G. F. , de Mello C. F. 
Published: October 2004

AbstractSummary: Although the precise mechanisms underlying the CNS degeneration of patients with glutaryl‐CoA dehydrogenase (GCDH) deficiency are still the subject of intense debate, many studies have highlighted that excitotoxicity plays a fundamental role in the neuropathology of this disease, particularly involving the N‐methyl‐D‐aspartate receptor subtype of ionotropic glutamate receptors. Modulation of the glutamatergic system by these compounds involves an inhibition of glutamate uptake into synaptosomes and synaptic vesicles, and a decrease in glutamate binding. Furthermore, glutaric and 3‐hydroxyglutaric acids inhibit glutamate decarboxylase, the key enzyme of GABA synthesis, and striatal GABAergic medium‐spiny neurons are highly vulnerable to 3‐hydroxyglutaric acid‐induced neurotoxicity. In conclusion, glutaric acid and 3‐hydroxyglutaric acid induce an imbalance in glutamatergic and GABAergic neurotransmission.
Vascular dysfunction as an additional pathomechanism in glutaric aciduria type I
Authors: Mühlhausen C. , Ergün S. , Strauss K. A. , Koeller D. M. , Crnic L. , Woontner M. , Goodman S. I. , Ullrich K. , Braulke T. 
Published: October 2004

AbstractSummary: The metabolic hallmark of glutaric aciduria type I (GA I) isthe deficiency of glutaryl‐CoA dehydrogenase (GCDH) with subsequent accumulation of glutaric acid, 3‐hydroxglutaric acid (3‐OH‐GA) and glutaconic acid. Current concepts regarding pathomechanisms of GA I focus on investigations of excitotoxic effects of 3‐OH‐GA. To identify pathogenetically relevant genes, microarray analyses were performed using brain material from GCDH‐deficient (GCDH−/−) and control mice. These microarray data confirmed recent pathogenic models, but also revealed alterations in genes that had previously not been correlated to the disease, e.g. genes concerning vascular biology. Subsequent in vitro and in vivo experiments confirmed direct effects of 3‐OH‐GA on vascular permeability and endothelial integrity. Clinical observations underscore the involvement of vascular dysfunction. In MRI scans of GA I patients, subdural effusions as well as dilated transarachnoid vascular plexuses were detected independently of encephalopathic crises. In fact, some of these findings are already detectable shortly after birth. MRI scans of a GA I patient performed during an acute encephalopathic crisis detected a dilated intrastriatal vasculature with perivascular hyperintensity, indicating local extravasation. In conclusion, we hypothesize that 3‐OH‐GA affects prenatal development of vessels, thus leading to an increased vulnerability of endothelial structures and subsequent vascular dysfunction. These observations display an additional pathomechanism in GA I and might explain frontotemporal hypoplasia and chronic subdural effusions in this disease. Elucidation of the pathomechanisms of vascular dysfunction may give further insights into the pathogenesis of GA I.
Challenges for basic research in glutaryl‐CoA dehydrogenase deficiency
Authors: KÖlker S. , Strauss K. A. , Goodman S. I. , Hoffmann G. F. , Okun J. G. , Koeller D. M. 
Published: October 2004

AbstractSummary: During the last decades, efforts have been made to elucidate the complex mechanisms underlying neuronal damage in glutaryl‐CoA dehydrogenase deficiency. A combination of in vitro and in vivo investigations have facilitated the development of several hypotheses, including the probable pathogenic role of accumulating glutaric acid and 3‐hydroxyglutaric acid. However, there are still many shortcomings that limit an evidence‐based approach to treating this inborn error of metabolism. Major future goals should include generation of a suitable animal model for acute striatal necrosis, investigation of the formation, distribution and exact intra‐ and extracellular concentrations of accumulating metabolites, a deeper understanding of striatal vulnerability, and systematic investigation of effects on cerebral gene expression during development and of the modulatory role of inflammatory cytokines.
Correlation of genotype and phenotype in glutaryl‐CoA dehydrogenase deficiency
Authors: Christensen E. , Ribes A. , Merinero B. , Zschocke J. 
Published: October 2004

AbstractSummary: We have investigated the correlation between genotype and phenotype in a large number of patients with glutaric aciduria type I (GA I). The deficiency of glutaryl‐CoA dehydrogenase has been confirmed in the Rigshospitalet's laboratory in 215 patients since 1975. Most of the patients were of European ancestry. Complete absence of enzyme activity was found in more than half of the patients, while 34% of patients had a residual activity up to 5% and a few patients had a residual activity of 5–15%. In four exceptional cases, a very high residual activity of up to 30% was found. Enzyme studies are thus a reliable method for confirming the diagnosis of GA I, although it may be difficult to distinguish exceptional ‘mild’ cases from heterozygous carriers for GA I. Three of the patients with very high residual activity are compound heterozygous for the missense mutations R227P and V400M, both of which are associated with residual enzyme activity of 8–10% in homozygous patients. Patients with a mild mutation on at least one chromosome frequently show unusual biochemical findings such as low or normal urinary excretion of glutaric acid and mild or only slightly increased excretion of 3‐hydroxyglutaric acid. In contrast, patients with severe mutations such as R402W or A293T on both alleles have no residual activity and show the typical urinary metabolite pattern. Clinical data were available for a subgroup of 79 patients. No correlation with the biochemical phenotype or the genotype could be established.
Reduction of lysine intake while avoiding malnutrition—Major goals and major problems in dietary treatment of glutaryl‐CoA dehydrogenase deficiency
Authors: Müller E. , KÖlker S. 
Published: October 2004

AbstractSummary: Treatment in glutaryl‐CoA dehydrogenase deficiency, an inborn error of metabolism of lysine and tryptophan, is mainly based on restriction of lysine intake, supplementation of carnitine, and an intensification of therapy during intercurrent illnesses. The major principle of dietary treatment is to reduce the production of glutaric acid and 3‐hydroxyglutaric acid by restriction of natural protein in general and of lysine in particular. In parallel to development, the growing child learns to utilize different protein sources, shifting the primarily milk‐based diet to a mixed diet. The changes in nutritional demands and food composition during the first years of life greatly influence nutritional support for affected patients at different ages. This article highlights frequent pitfalls of dietary treatment for this disease and focuses on particular risks of malnutrition in terms of essential amino acids and micronutrients and/or excess intake of lysine between age 3 months and age 6 years. We conclude from the examples given that restriction of natural protein intake plus application of lysine‐free amino acid mixtures minimizes the risk of malnutrition and allows a reliable control of protein and lysine intake and, thus, seems particularly recommendable during the vulnerable period for acute encephalopathic crises. The efficacy of these theoretical and experience‐based approaches to dietary treatment of glutaryl‐CoA dehydrogenase deficiency should be investigated in detail in prospective clinical studies.
Management of movement disorders in glutaryl‐CoA dehydrogenase deficiency: Anticholinergic drugs and botulinum toxin as additional therapeutic options
Authors: Burlina A. P. , Zara G. , Hoffmann G. F. , Zschocke J. , Burlina A. B. 
Published: October 2004

AbstractSummary: Glutaric aciduria type I is an inborn error of metabolism due to the deficiency of glutaryl‐CoA dehydrogenase, an enzyme responsible for the catabolism of lysine, hydroxylysine and tryptophan. The most important neurological symptoms include dyskinesia and dystonia, which can be focal, segmental or generalized. Treatment of the extrapyramidal syndrome is often unsatisfactory. We report our experience in the treatment of generalized and focal dystonia with anticholinergic drugs and botulinum toxin type A, respectively. Both therapies proved beneficial.
Glutaric aciduria type I: Outcome in the Republic of Ireland
Authors: Naughten E. R. , Mayne P. D. , Monavari A. A. , Goodman S. I. , Sulaiman G. , Croke D. T. 
Published: October 2004

AbstractSummary: Twenty‐one patients have been diagnosed with glutaric aciduria type I over a 16‐year period in the Republic of Ireland, 11 following clinical presentation and 10 following a high‐risk screen. Nineteen have been managed with diet. Eight patients have died, of whom 7 were diagnosed clinically. Six had dystonic and one spastic cerebral palsy. Of the 11 patients who did not have cerebral palsy, 10 were diagnosed following a high‐risk screen. Seven of the 11 have no abnormal neurological signs; 6 of the 7 have abnormal CT or MRI findings; and no case of striatal degeneration has occurred during the past 14 years in the high‐risk screened group.
Raised HDL cholesterol in Fabry disease: Response to enzyme replacement therapy
Authors: Cartwright D. J. , Cole A. L. , Cousins A. J. , Lee P. J. 
Published: October 2004

AbstractSummary: Fabry disease patients have increased risk of vascular disease despite cardioprotective increased HDL‐cholesterol. Enzyme therapy does not significantly alter the lipid profile in the short term.
Reciprocal regulation of glutathione S-transferase spliceforms and the Drosophila c-Jun N-terminal kinase pathway components
Authors: UDOMSINPRASERT Rungrutai , BOGOYEVITCH Marie A. , KETTERMAN Albert J. 
Published: October 2004

In mammalian systems, detoxification enzymes of the GST (glutathione S-transferase) family regulate JNK (c-Jun N-terminal kinase) signal transduction by interaction with JNK itself or other proteins upstream in the JNK pathway. In the present study, we have studied GSTs and their interaction with components of the JNK pathway from Diptera. We have evaluated the effects of four Delta class Anopheles dirus GSTs, GSTD1-1, GSTD2-2, GSTD3-3 and GSTD4-4, on the activity of full-length recombinant Drosophila HEP (mitogen-activated protein kinase kinase 7; where HEP stands for hemipterous) and the Drosophila JNK, as well as the reciprocal effect of these kinases on GST activity. Interestingly, even though these four GSTs are alternatively spliced products of the same gene and share &gt;60% identity, they exerted different effects on JNK activity. GSTD1-1 inhibited JNK activity, whereas the other three GST isoforms activated JNK. GSTD2-2, GSTD3-3 and GSTD4-4 were inhibited 50–80% by HEP or JNK but GSTD1-1 was not inhibited by JNK. However, there were some similarities in the actions of HEP and JNK on these GSTs. For example, binding constants for HEP or JNK inhibiting a GST were similar (20–70 nM). Furthermore, after incubation of the GSTs with JNK, both JNK and the GSTs changed catalytic properties. The substrate specificities of both GSTs and JNK were also altered after their co-incubation. In addition, glutathione modulated the effects of JNK on GST activity. These results emphasize that different GST spliceforms possess different properties, both in their catalytic function and in their regulation of signalling through the JNK pathway.
Significance of the endothelin ETA receptor in the haemodynamic and inotropic effects of endothelin-1 in rats
Authors: BEYER Martin E. , HÖVELBORN Tobias , DELABAR Ursula , HOFFMEISTER Hans Martin 
Published: October 2004

The main aim of the present study was to investigate the direct inotropic effects of stimulation of the endothelin (ET) receptor ETA under in vivo conditions. It is well known that ETA receptor stimulation causes pronounced vasoconstriction. The ET-1-induced coronary vasoconstriction may lead to myocardial ischaemia and, consequently, to cardiodepressor effects that may mask the direct positive inotropic effect of ETA receptor stimulation. Thus, in the present study, steps were taken to avoid this possibility. In anaesthetized open-chest rats the haemodynamic and inotropic effects of ETA receptor stimulation were studied by monitoring responses evoked by ET-1 (1 nmol/kg of body weight) after ETB receptor blockade with BQ 788 (0.5 μmol/kg of body weight); these responses were compared with saline controls (after ETB receptor blockade). To avoid vasoconstrictor effects induced by ETA receptor stimulation, additional experiments were performed in the presence of the vasodilator adenosine (2.0 mg·kg−1 of body weight·min−1). Myocardial function was also examined during aortic clamping so as to circumvent the effect of changes in afterload. We studied further the effect of ETA receptor stimulation on myocardial energy metabolism. ETA receptor stimulation reduced cardiac output (−49% compared with control), raised total peripheral resistance (+173%) and reduced myocardial ATP content (−23%). Aortic clamping did not reveal a positive inotropic effect of ETA receptor stimulation. Furthermore, even though adenosine attenuated the decrease in cardiac output (−21%), the increase of total peripheral resistance (+48%) and prevented the fall of myocardial ATP content (+6%), this did not unmask a positive inotropic effect of ETA receptor stimulation. Thus we conclude that ETA receptor stimulation causes vasoconstriction and myocardial ischaemia, but has no positive inotropic effects in rats.
SEASONAL FLUCTUATIONS IN TISSUE NITROGEN, PHOSPHORUS, AND N:P FOR FIVE MACROALGAL SPECIES COMMON TO THE PACIFIC NORTHWEST COAST1
Authors: Wheeler Patricia A. , Björnsäter Bo R. 
Published: October 2004

ABSTRACTVariations in tissue nitrogen (N) and phosphorus (P) were examined over a complete seasonal cycle for five macroalgae common in Oregon coastal water. Tissue N ranged from 2.0 to 5.5% dry weight (dry wt) in leafy macroalgae (Enteromorpha intestinalis (Linnaeus) Link. Ulva fenestrata Postels et Ruprecht, and Porphyra sp.) and from 0.9 to 2.6% drt wt in branched macroalgae (Codium fragile (Suringar) Hariot and Pelvetiopsis limitata Setchell). Tissue P ranged from 0.32 to 0.86% dry wt in leafy macroalgae and from 0.27 to 0.50% dry wt in branched macroalgae, Ulva fenestrata, C. fragile, and P. limitata appeared to be N limited during part of the year based on tissue N levels. Variations in N:P showed a more distinct seasonal pattern than either tissue N and tissue P. All macroalgae examined appeared to be N limited at least part of the year based on N:P composition, and P limitation occurred for all macroalgae examined except C. fragile. Our results suggest that tissue N:P ratio for macroalgae may be a good index for evaluating in situ nutrient status.
PHOTOSYNTHETIC RESPONSE OF NATURAL ASSEMBLAGES OF MARINE BENTHIC MICROALGAE TO SHORT‐AND LONG‐TERM VARIATIONS OF INCIDENT IRRADIANCE IN BAFFIN BAY, TEXAS1
Authors: Blanchard Gérard F. , Montagna Paul A. 
Published: October 2004

ABSTRACTThis study was designed to understand the high variability characterizing primary production rates of microphytobenthos. The photosynthetic efficiency (αB) and photosynthetic capacity (PBmax) of the microphytobenthos were measured at different times of the day on two different dates (8 May and 7 July 1990). In July, unusually low light conditions were caused by the development of a brown tide (chrysophytes). Both light‐limited and light‐saturated photosynthesis changed at hourly and monthly scales. There was a linear relationship between αB and PBmax, suggesting a common response to environmental factors [αB= 0.0075(±0.00063)·PBmax+ 0.00097(±0.0071), R2= 0.94]. Incident irradiance at the sediment‐water interface was the primary physical factor that explained variability of both αB (84%) and PBmax (92%). Temperature had a negative but minor effect that explained an extra 8% and 2% of the variance, respectively. There was no diel rhythm of αB and PBmax and incident irradiance was regulated by wind‐induced currents. Therefore, microphytobenthos photosynthesis seemed to be primarily controlled by wind events in Baffin Bay.
SIMAZINE‐INDUCED INHIBITION IN PHOTOACCLIMATED POPULATIONS OF ANABAENA CIRCINALIS (CYANOPHYTA)1
Authors: Millie David F. , Hersh C. Mark , Dionigi Christopher P. 
Published: October 2004

ABSTRACTThe effects of the triazine herbicide, simazine, on photosynthetic oxygen evolution and growth rate in photoacclimated populations of Anabaena circinalis Rabenhorst were investigated. Chemostat populations were acclimated to photon flux densities (PFDs) of 50, 130, and 230 μmol·m−2·s−1 of photosynthetic active radiation (PAR), Decreases in chlorophyll a (Chl a). c‐phycocyanin (CPC), and total carotenoid (TCar) contents and CPC: Chl a and CPC: TCar ratios of populations coincided with increasing PFD, Polynomial regression models that characterize inhibition of photosynthesis for populations acclimated to 50 and 130 μmol photons·m−2·s−1 PAR were distinct from the model for populations acclimated to 230 μmol photons·m−2·s−1 PAR. Simazine concentrations that, depressed oxygen evolution 50% compared to controls decreased with increasing PFD. Increases and decreases in both biomass and growth rate coincided with increasing PFD and simazine concentration, respectively. Simazine concentrations that depressed growth rate 50% compared to controls increased with decreasing PFD. The differences in photosynthetic and growth inhibition among photoacclimated populations indicate that sensitivity to photosystem II inhibitors is affected by alterations in pigment contents.