bluuebunny/crossref_metadata_2025 huggingface dataset:
Synthesis, Crystal Structures and Properties of Na2ReO3 and of a Second Modification of Na5ReO6
Authors: Sobotka Bettina M. , Mudring Anja‐Verena , Möller Angela
Published: October 2004

Single crystals of Na5ReO6 have been obtained from the reaction of Na2O, rhenium metal and CdO in Ag containers at 550 °C. At 700 °C Na2ReO3 is formed possibly via a topotactic redox reaction of Na5ReO6 with rhenium metal. Here we report the crystal structures of Na2ReO3 and a second modification of Na5ReO6, ex‐situ X‐ray diffraction studies of the reactivity within the system Na / Re / O, selected spectroscopic investigations of the two compounds and Extended‐Hückel calculations of the [Re2O6]4‐ anion, containing a rhenium‐rhenium triple‐bond.
HN(C3N3Cl2)2 und N(C3N3Cl2)3 ‐ mögliche molekulare Zwischenstufen bei der Synthese von Kohlenstoff(IV)‐nitriden
Authors: Tragl Sonja , Gibson Katharina , Meyer H.‐Jürgen
Published: October 2004

Bei der Reaktion von Cyanurchlorid mit Melamin wurde die Bildung der Amine Bis‐(4, 6‐dichloro‐[1, 3, 5]triazin‐2‐yl)amin HN(C3N3Cl2)2 und Tris‐(4, 6‐dichloro‐[1, 3, 5]triazin‐2‐yl)amin N(C3N3Cl2)3 beobachtet. Der Nachweis dieser Verbindungen erfolgte durch Einkristalldiffraktometrie bei Raumtemperatur. HN(C3N3Cl2)2 konnte hierbei als Addukt mit einem Mol Cyanurchlorid pro Formeleinheit isoliert werden. HN(C3N3Cl2)2 · C3N3Cl3 kristallisiert in der Raumgruppe C 2/c mit den Gitterkonstanten a = 739, 3(1), b = 1365, 5(2), c = 1700, 7(3) pm und β = 95, 86(2)°. Die Kristallstruktur von N(C3N3Cl2)3 stimmt mit der von Yaghi et al. bei 158 K bestimmten überein. Aufgrund der höheren Messtemperatur wurden etwas größere Gitterkonstanten erhalten (Raumgruppe R{\bar 3}$; a = 1933, 5(2) und c = 758, 41(7) pm).

Auch bei Reaktionen von Cyanurchlorid mit Lithiumnitridoborat bzw. Lithiumcarbodiimid entstanden Einkristalle von N(C3N3Cl2)3. Dieses Molekül ist möglicherweise eine Zwischenstufe bei der Bildung von polymeren Verbindungen mit der Zusammensetzung C3N4, deren Strukturen aus Triazin‐Ringen aufgebaut sind, die über dreibindige N‐Atome verknüpft sind.
Structure Prediction of Inorganic Nanoparticles with Predefined Architecture using a Genetic Algorithm
Authors: Woodley Scott M. , Sokol Alexey A. , Catlow C. Richard A.
Published: October 2004

A multistage scheme has been developed for the structure prediction of viable clusters, where a genetic algorithm is implemented within the first stage to generate approximate structures that can be refined via direct energy minimisation techniques in a later stage. Inclusion and exclusion zones, regions of space where atoms are allowed and forbidden, respectively, are used to reduce the search space and to steer the predicted structures so that they have a predefined architecture, e.g. bubble‐like as oppose to dense phase‐like. In a final stage, electronic, rather than atomistic, calculations were performed. The method has successfully generated sets of stable II‐VI semiconductor clusters (MX)n, with n ranging from 1 to 15. This method allows one to predict different types of morphology that may not necessarily contain the global minimum structure, but are of particular importance for small clusters in nature and the laboratory.
Ein ionenleitendes Natriumnickelborat, Na2Ni2B12O21, mit offener Kanalstruktur
Authors: Pompetzki M. , Albert B.
Published: October 2004

Na2Ni2B12O21wurde durch eine Festkörperreaktion bei 800 °C aus Ni3(BO3)2, Na2B4O7und H3BO3dargestellt. Die Kristallstruktur wurde auf Basis von Röntgenpulverdaten mit derRietveld‐Methode verfeinert (I2/a,a= 17, 0662(4) Å,b= 4, 4801(1) Å,c= 19, 4223(5) Å,β= 102, 907(2)°,Rp= 4, 71 %,Rwp= 6, 44 %). Bei der Struktur handelt es sich um ein dreidimensionales B‐O‐Netzwerk mit darin eingebetteten, aus dimeren, kantenverknüpften, verzerrten NiO6‐Oktaedern bestehenden Baugruppen. Das Netzwerk weist eindimensionale Kanäle längs der kristallographischenb‐Achse auf. Natriumionen in diesen Kanälen werden bei Temperaturen oberhalb 480 °C mobil. Dies kann anhand von Messungen der elektrischen Leitfähigkeit nachgewiesen werden, es handelt sich bei der Titelverbindung somit um einen festen Elektrolyten.
Synthese, IR‐Spektren und Kristallstrukturen der Diphenylchalkogeno‐triphenylphosphiniminium‐Salze [Ph2ENPPh3]Cl (E = S, Se)
Authors: Neumüller Bernhard , Dehnicke Kurt
Published: October 2004

Die Diphenylchalkogeno‐triphenylphosphiniminium‐Salze [Ph2ENPPh3]Cl (E = S, Se) wurden durch oxidative Addition von N‐chloro‐triphenylphosphanimin an Diphenylsulfid bzw. Diphenylselenid in Dichlormethanlösung hergestellt. Nach den IR‐Spektren und den Kristallstrukturen bilden beide Verbindungen ionische Strukturen. Der relativ kurze Se···Cl‐Abstand der Selenverbindung wird als Ionenpaar mit einer verzerrt Ψ‐trigonal‐bipyramidalen Koordination am Selenatom interpretiert, wobei das Chloratom und das Stickstoffatom die Apical‐Positionen besetzen.

[Ph2SNPPh3]Cl (1): Raumgruppe P21, Z = 2, Gitterkonstanten bei 193 K: a = 868, 8(1); b = 1466, 6(2); c = 1008, 1(1) pm; β = 97, 64(1)°; R1 = 0, 026.

[Ph2SeNPPh3]Cl (2): Raumgruppe P21, Z = 2, Gitterkonstanten bei 193 K: a = 892, 2(1); b = 1443, 1(1); c = 1011, 4(1) pm; β = 98, 52(1)°; R1 = 0, 022.
The First Palladium Tin Selenide: Pd7Sn2Se9.85
Authors: Pocha R. , Johrendt Dirk
Published: October 2004

Pd7Sn2Se9+x with x = 0.5‐0.85 were prepared by heating the elements at 750 °C in silica ampoules under argon atmosphere. The crystal structures were determined by single crystal X‐ray methods. The selenides crystallize in a new hexagonal structure type (Pd7Sn2Se9.85: P63/mmc, Z = 2, a = 7.297(2), c = 16.314(3) Å) with palladium atoms in six‐fold octahedral and four‐fold square coordination of selenium. Layers of isolated PdSe6 octahedra are connected along [001] by face‐ and edge‐sharing PdSe4/2 squares, whose Se atoms form anti‐cuboctahedra, each centered by selenium atoms with fractional occupations from x = 0.513(5) up to x = 0.847(6). The lattice parameter c increases linearly with x from 16.167(3) Å for Pd7Sn2Se9.51 to 16.314(3) Å in Pd7Sn2Se9.85. Tin is bonded to three selenium and three palladium atoms, respectively, both together form a distorted trigonal antiprism. Measurements of the electrical conductivity and the magnetic susceptibility reveals metallic Pauli‐paramagnetic behavior. The electronic charge distribution based on quantum mechanical calculations within the AIM theory is discussed. A mechanism based on the balance between increasing bonding energy and reorganization of charges is proposed and suspected to be a reason for the fractional occupation of one selenium site.
Chiral Recognition among Tris(diimine)‐metal Complexes. 9. Crystal Structure Prediction for the Chiral Molecular Salt [Ru(bpy)3](PF6)2
Authors: Huttner Dominikus , Breu Josef
Published: October 2004

Crystal structure prediction has been carried out for the chiral molecular ionic compound [Ru(bpy)3](PF6)2. The experimentally observed structures (enantiomerically pure and racemic, respectively) could be identified in the simulations. Furthermore, the calculations confirmed the experimental finding that this compound does not resolve spontaneously into the enantiomers upon crystallization (conglomerate crystallization).

Additionally, two energetically sensible crystal structures with promising packing motifs could be identified. From an energetic point of view, these should be accessible as metastable Ostwald products.
Darstellung und Struktur von Sc3(Sc0, 31Al0, 69)O6 ‐ einem neuen Abkömmling des Mg3TeO6‐Strukturtyps
Authors: Müller Daniel , Assenmacher Wilfried , Mader Werner
Published: October 2004

Sc3(Sc0, 31Al0, 69)O6 wurde als farbloses Pulver sowohl über eine Festkörperreaktion aus den binären Oxiden und auch phasenrein über die Sol‐Gel‐Methode hergestellt. Durch die Kombination von Röntgen‐Pulverdiffraktometrie, Elektronen‐Einkristallbeugung und energiedispersiver Röntgenspektroskopie wurde die Verbindung identifiziert, die Raumgruppe bestimmt und die Kristallstruktur nach der Rietveld‐Methode verfeinert. Sc3(Sc0, 31Al0, 69)O6 (Raumgruppe $R{\bar 3}$, Nr. 148, a = 877, 45(1) pm, c = 1013, 33(1) pm, Z = 6) kristallisiert im Mg3TeO6‐Strukturtyp, wobei eine statistische Besetzung der Kationen auf den dreizähligen Lagen erstmals für diesen Strukturtyp beobachtet wird.
Erucamide
Authors: Molnar N. M.
Published: October 2004

Erucamide has, to a great extent, replaced the use of oleamide because of its higher mp and higher heat resistance. These properties are desirable because of the higher operating temperatures of newer polymers. Various derivatives are discussed, such as N‐stearyl erucamide, which has a still higher mp and greater thermal stability. This type of product is used principally as a slip additive, an antiblock agent, and for paper‐coating compositions and water‐proofing. Erucamide and some of its derivatives are approved for use in polymers at low concentration levels. At these levels, the material is effective without adversely affecting other physical characteristics of the polymer. A summary of the literature is presented, including a review of some of the patents. Size of the market also is discussed.
A coulometric method for the determination of low peroxide values of fats and oils
Authors: Fiedler Ulla
Published: October 2004

The AOCS official method for the determination of peroxide values of fats and oils has been modified to perform more precise analyses, especially in the range of extremely low values (peroxide value <1). In the newly developed method presented here, the final titrimetric step of an iodometric method has been replaced by an electrochemical technique, based upon coulometric reduction of the iodine at an electrode maintained at a controlled potential. Using this method peroxide values down to 0.06 were determined with reasonable precision. Finally, the influence of different parameters on the peroxide value obtained was investigated.
Determination of emulsion stability by microwave irradiation
Authors: Petrowski Gary E.
Published: October 2004

The stability of emulsions has been determined by a microwave irradiation method. After this treatment, the surface temperature of the emulsion tends to be greatest and the temperature difference between the surface and the bottom of the emulsion smallest for the more stable emulsions. These results have been explained in terms of the nature of microwave heating and a partially destabilized emulsion.
Brief report: Psychological characteristics of less severe forms of eating disorders: an epidemiological study among 259 female adolescents
Authors: Cotrufo Paolo , Gnisci Augusto , Caputo Immacolata
Published: October 2004

We screened a sample of 259 female students, aged 17–20 years old, by means of the Eating Disorder Inventory 2 (EDI 2) and the General Health Questionnaire (GHQ‐28). Those students identified to be at risk for an eating disorder underwent a semi‐structured interview to provide a diagnosis according to DSM‐IV criteria. We also considered the prevalence of “not full‐blown diagnosis”. We found two cases of full‐syndrome (0.77%), both bulimics, 9 partial‐syndrome bulimia nervosa (3.47%) and 1 of partial‐syndrome binge eating disorder (0.38%). Moreover, 18 (6.94%) girls met the criteria for subclinical‐syndrome, of which the majority was subclinical anorexia (5.79%). We did not find statistically relevant differences between the anorexic and the bulimic samples on the psychological characteristics measured by the EDI 2 subscales. These findings suggest the hypothesis that anorexia and bulimia could have the same psychopathological core and bulimia could be considered as a “failed” anorexia.
Cough headache: frequency, characteristics and the relationship with the characteristics of cough
Authors: Özge Cengiz , Atiş Sibel , Özge Aynur , Duce Meltem Nass , Saraçoǧlu Mehmet , Saritaş Emel
Published: October 2004

Cough headache (CH) is a relatively rare, but an important complication of cough. The aim of this cross‐sectional clinical study was to evaluate the frequency, characteristics and etiology of CH among the patients referred to our Outpatient Department with the complaint of cough, and to investigate the relationship between their cough and headache characteristics. We evaluated 96 females and 69 males, a total of 165 patients with cough. Among those, 57 patients (34.5%) had one or more cough complications and 32 patients (19.3%) were diagnosed as CH. Although it was known that most of the patients with CH had benign headache characteristics, the ratio of the symptomatic CH was not low (37.5% of the CH patients and 7% of patients with cough). Also, there was a significant correlation between the frequency of cough and the severity of headache. Logistic regression analysis showed that the incidence of CH was increased 0.4‐fold, when frequency of cough increased. Age, sex, tobacco use ad the duration of cough were not found to be predictive factors for CH. Logistic regression analysis showed that the rate of cough complications increased 2.08‐fold, when the duration of cough was longer than eight weeks (p = 0.03) and 0.4‐fold when the frequency of cough increased (p = 0.02). In conclusion, CH is a relatively rare, but an important complication of cough and it commonly has an effective treatment available. Radiological work‐up was crucial in ruling out other causes of headache and to confirm that the CH was truly benign.
The service volunteer – loyalty chain: an exploratory study of charitable not‐for‐profit service organizations
Authors: Wisner Priscilla S. , Stringfellow Anne , Youngdahl William E. , Parker Lenore
Published: October 2004

Many not‐for‐profit organizations rely on volunteers to help accomplish their service objectives. Although volunteers work alongside or in some cases replace employees in the delivery of service, incorporating volunteer labor into the service delivery system of the not‐for‐profit poses unique challenges. Understanding these challenges represents an important foundation‐building step in understanding the implications for service design and service operations when using volunteers. This paper identifies and describes service design and operational factors relevant to volunteer satisfaction in not‐for‐profit organizations. Using data collected from 288 volunteers working in 43 not‐for‐profit agencies, the study explores the elements of service delivery that impact volunteer satisfaction, and further tests the relationship between volunteer satisfaction and loyalty to the not‐for‐profit organization. Findings include that satisfied volunteers are more likely to remain for longer periods of time with the same organization, are more likely to donate financially to the not‐for‐profit, and are more likely to recommend the volunteer experience to others. Each of these outcomes helps to ensure the continued sustainability of the not‐for‐profit organization.
Judith Rich Harris: The Miss Marple of Developmental Psychology
Authors: Wesseling Elisabeth
Published: October 2004

Argument

This paper contributes to inquiries into scientific personae by employing a rhetorical approach. It analyzes the persuasive strategies of Judith Rich Harris in The Nurture Assumption: Why Children Turn Out the Way They Do (1998). Rhetorical analysis of Harris' self-fashioning in this remarkable best-seller and the reactions of the press to her persona demonstrates the resilience of specific archaic cultural repertoires for constructing scientific identities. While historical studies investigate how repertoires for scientific self-fashioning evolve through time, rhetoric reveals how identity models from an earlier age may be appropriated in the present.
Mathematicians Forced to Philosophize: An Introduction to Khinchin's Paper on von Mises' Theory of Probability
Authors: Siegmund-Schultze Reinhard
Published: October 2004

What follows shall provide an introduction to a predominantly philosophical and polemical, but historically revealing, paper on the foundations of the theory of probability. The leading Russian probabilist Aleksandr Yakovlevich Khinchin (1894–1959) (see fig. 1) wrote the paper in the late 1930s, commenting on a slightly older, but still competing approach to probability theory by Richard von Mises. Together with the even more influential Andrey Nikolayevich Kolmogorov (1903–1987), who was nine years his junior, Khinchin had revolutionized probability theory around 1930 by introducing the modern measure-theoretic approach, which is still standard today and which allowed for a sufficiently general treatment of important new notions such as “stochastic processes.” This development had its first culmination in Kolmogorov's booklet, Grundbegriffe der Wahrscheinlichkeitsrechnung, written in German in 1933, which has exerted an enormous influence world wide.
Convention on the Law of the Non-Navigational Uses of International Watercourses
Authors: Fitzmaurice Malgosia
Published: October 2004

On 11 April 1997, the text of the Convention on the Law of the Non-Navigational Uses of International Watercourses was presented by the Working Group of the Whole (WG) of the United Nations General Assembly Sixth Committee to the United Nations General Assembly (UNGA). This Convention is based on the 1994 Draft Articles on the same topic prepared by the International Law Commission (ILC). These Draft Articles were approved on second reading by the ILC during its 46th session in 1994 and subsequently submitted to the 49th session of the UNGA in 1994 for consideration by states. By its Resolution 49/52, the UNGA invited states to present written submissions to comment on the Draft Articles and at the same time it proposed that a working group on the whole of the UNGA Sixth Committee be established to convene during the 51st session of UNGA (September-December 1996) to elaborate the text for a convention. During its first session, the WG did not manage to accomplish this task. The final text submitted to the UNGA on 11 April 1997 was the result of the second session of the WG which had deliberated during the period from 24 March to 4 April 1997.
Justifiable Doubts as to the Arbitrator's Impartiality or Independence
Authors: van den Berg Albert Jan
Published: October 2004

Traditionally, most arbitration acts provided that an arbitrator could be challenged on the same grounds as those on which judges could be challenged. Thus, arbitrators could be challenged on specific grounds, such as a family relationship with one of the parties or animosity towards one of the parties. The more modern approach is to provide for an open norm for the grounds on which challenges can be brought. This approach finds its origin in the UNCITRAL Arbitration Rules adopted in 1976. It provides that: “[a]n arbitrator may be challenged only if circumstances exist that give rise to the justifiable doubts as to his impartiality or independence”. This standard was received in many other arbitration acts and rules. The standard nowadays being more or less uniform, its interpretation and application are far from easy.
The Case Concerning Oil Platforms (Islamic Republic of Iran v. United States of America) – Preliminary Objection
Authors: Clark Roger S.
Published: October 2004

The case-law of the International Court of Justice (Court) is replete with arguments about whether the Court has jurisdiction to entertain the particular dispute (or request for advisory opinion) with which the Court is faced. These arguments are framed at one level as matters of interpretation of the relevant instruments. But they typically play out as well a multiplicity of variations on the overlapping themes of sovereignty (the extent to which states have been prepared to concede decision-making to third-party settlement mechanisms) and justiciability (the extent to which they will accept that an issue may be governed by ‘law’ and thus be susceptible to resolution by judicial actors).
The European Union institutions in the draft Constitution for Europe
Authors: VAN GERVEN WALTER
Published: October 2004

This paper deals with the Institutions of the European Union in the draft Treaty establishing a Constitution for Europe (hereinafter: ‘the draft Constitution’) submitted to the European Council meeting in Thessaloniki on 20 June 2003. It describes these institutions and their task from a perspective of the Union's democratic legitimacy. The paper is based on a book entitled The European Union: a Polity of States and Peoples, which will be published by Stanford University Press and Hart Publishing, Oxford. In this book, I examine the democratic legitimacy of the European Union as a whole. The book parts from the proposition that the Union is a ‘body politic’ which develops into a federal system, however not a State, with a parliamentary consensual (non-majoritarian) form of government. In the meantime, the draft Treaty has been amended by the Inter-Governmental Conference (IGC) held in Brussels on 17/18 June 2004. In so far as the amendments relate to the subject of this paper, they are mentioned below in the text or the endnotes.
Decoding Chomsky
Authors: KNIGHT CHRIS
Published: October 2004

Noam Chomsky is an enigma. To many, he is — and has been for 50 years — the most prominent and courageous academic opponent of his country's militarist ambitions around the globe. Yet among those who admire him on that score, few find it easy to relate to his seemingly obscure theories about language. The academic community acclaims Chomsky as the principal inspiration behind the so-called ‘cognitive revolution’ in psychology and related sciences — an intellectual development reflecting corporate pressures and initially sponsored by the United States military. This article investigates the paradoxical relationship between Chomsky's political activism and his science.
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 Fragmente

When 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.
WHY CONSTANT? A CRITICAL OVERVIEW OF THE CONSTANT REVIVAL
Authors: ROSENBLATT HELENA
Published: October 2004

Recent years have seen a remarkable renewal of interest in the thought of Benjamin Constant (1767–1830). For long recognized as the author of the literary masterpiece Adolphe, Constant is now receiving increasing attention for his political writings. Paperback editions of his major works are presently available in both French and English, helping to establish his growing reputation as a founding father of modern liberalism. Constant's stature as a seminal liberal thinker has benefited from the recent climate of opinion in the Western world and, in particular, from the return to fashion of liberalism as a social and political doctrine. Paradoxically, however, this political climate has also led to some problems, since presentist concerns have left an undeniable imprint on the image we have of Constant.
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

Summary: 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

Summary: 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

Summary: 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

Summary: 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

Summary: 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

Summary: 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

Summary: 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

Summary: 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

Summary: 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

Summary: 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

Summary: 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.
High‐resolution maps of the sea‐ice concentration from MODIS satellite data
Authors: Drüe Clemens , Heinemann Günther
Published: October 2004

For realistic simulations of the atmosphere‐sea‐ice‐ocean interaction in polar regions, maps of the sea‐ice concentration of up to one kilometer resolution are needed. In the present paper, an algorithm is described, which is able to derive such maps based on satellite data from MODIS (Moderate Resolution Imaging Spectroradiometer). This algorithm first retrieves sea‐ice concentration for each scene from the satellite‐sensed surface temperature. Then, data from multiple satellite overpasses within one day are combined to a map of the sea‐ice concentration. The remaining gaps are finally filled in by a scheme considering the brightness temperatures in the gap areas. The results are compared to operational sea‐ice concentration products based on SSM/I (special sensor microwave imager) and good agreement is found when averaging MODIS‐based data to the same resolution.
Understanding spaced‐receiver zonal velocity estimation
Authors: Ledvina B. M. , Kintner P. M. , de Paula E. R.
Published: October 2004

Spaced‐receiver analysis can be used to estimate the ionospheric zonal irregularity velocity. This involves first measuring the scintillation pattern velocity and then estimating the zonal irregularity velocity. A straightforward technique involving projection planes has been used to derive a relationship between the satellite position and velocity, the irregularities' position and velocity, and the scintillation pattern velocity. This relationship relies on the alignment of the irregularities along the field lines but is independent of the irregularity spectrum. When simplified, this relationship can be used to estimate the zonal irregularity velocity using either geostationary satellites or Global Positioning System (GPS) satellites. This estimation is straightforward but must be performed in a careful manner. An example of zonal irregularity velocity estimation using GPS satellites is presented. The average zonal irregularity velocity is estimated over a 3‐week period in March 2002 at São Luís, Brazil (−3.59° dip angle). This hourly average velocity has been compared with the climatological radar observations from Jicamarca Observatory. The spaced‐receiver zonal velocity agrees well (within ±8 m/s) with the radar's observations during postsunset hours.
Effects of Martian conditions on numerically modeled, cooling‐limited, channelized lava flows
Authors: Rowland Scott K. , Harris Andrew J. L. , Garbeil Harold
Published: October 2004

We used the FLOWGO thermorheological model to examine the effects of Martian gravitational and environmental conditions on the cooling‐limited behavior of lava flowing in a channel. The largest effect is due to the lower gravity on Mars as compared to Earth, which causes lava to flow more slowly. The lower velocity means that heat loss per distance down a Mars channel is greater even though the lower velocity also produces a higher percentage cover of insulating crust. Gravity alone causes the Mars flow to be >65 km shorter than an Earth flow with an equivalent volumetric flow rate. The cooler ambient Mars atmosphere causes a slight increase in heat loss by forced convection. This slows the flow a bit more, resulting in a very slight increase in heat loss per distance by all mechanisms, and decreases the modeled flow length by ∼1 km, a difference well below our model uncertainty. Replacing terrestrial values of atmospheric density, viscosity, thermal conductivity, heat capacity, and cubic expansivity makes convection less efficient and increases flow length by ∼15 km. Nevertheless, at the same volumetric flow rate, lava flows ∼50 km less far under Martian conditions than under terrestrial conditions. Our specific model flow has a volumetric flow rate of ∼5000 m3 s−1 and traveled ∼190 km down a channel on a constant 7° slope. This volumetric flow rate is slightly less than the maximum rates during the 1783–1785 Laki eruption and is 5–10 times greater than those of typical Mauna Loa eruptions.
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 >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.
Maturation of the unusual single-cysteine (XXXCH) mitochondrial c-type cytochromes found in trypanosomatids must occur through a novel biogenesis pathway
Authors: ALLEN James W. A. , GINGER Michael L. , FERGUSON Stuart J.
Published: October 2004

The c-type cytochromes are characterized by the covalent attachment of haem to the polypeptide via thioether bonds formed from haem vinyl groups and, normally, the thiols of two cysteines in a CXXCH motif. Intriguingly, the mitochondrial cytochromes c and c1 from two euglenids and the Trypanosomatidae contain only a single cysteine within the haem-binding motif (XXXCH). There are three known distinct pathways by which c-type cytochromes are matured post-translationally in different organisms. The absence of genes encoding any of these c-type cytochrome biogenesis machineries is established here by analysis of six trypanosomatid genomes, and correlates with the presence of single-cysteine cytochromes c and c1. In contrast, we have identified a comprehensive catalogue of proteins required for a typical mitochondrial oxidative phosphorylation apparatus. Neither spontaneous nor catalysed maturation of the single-cysteine Trypanosoma brucei cytochrome c occurred in Escherichia coli. However, a CXXCH variant was matured by the E. coli cytochrome c maturation machinery, confirming the proposed requirement of the latter for two cysteines in the haem-binding motif and indicating that T. brucei cytochrome c can accommodate a second cysteine in a CXXCH motif. The single-cysteine haem attachment conserved in cytochromes c and c1 of the trypanosomatids is suggested to be related to their cytochrome c maturation machinery, and the environment in the mitochondrial intermembrane space. Our genomic and biochemical studies provide very persuasive evidence that the trypanosomatid mitochondrial cytochromes c are matured by a novel biogenesis system.
Crystal structures of the antitermination factor NusB from Thermotoga maritima and implications for RNA binding
Authors: BONIN Irena , ROBELEK Rudolf , BENECKE Heike , URLAUB Henning , BACHER Adelbert , RICHTER Gerald , WAHL Markus C.
Published: October 2004

NusB is a prokaryotic transcription factor involved in antitermination processes, during which it interacts with the boxA portion of the mRNA nut site. Previous studies have shown that NusB exhibits an all-helical fold, and that the protein from Escherichia coli forms monomers, while Mycobacterium tuberculosis NusB is a dimer. The functional significance of NusB dimerization is unknown. We have determined five crystal structures of NusB from Thermotoga maritima. In three crystal forms the protein appeared monomeric, whereas the two other crystal forms contained assemblies, which resembled the M. tuberculosis dimers. In solution, T. maritima NusB could be cross-linked as dimers, but it migrated as a monomer in gel-filtration analyses, suggesting a monomer/dimer equilibrium with a preference for the monomer. Binding to boxA-like RNA sequences could be detected by gel-shift analyses and UV-induced cross-linking. An N-terminal arginine-rich sequence is a probable RNA binding site of the protein, exhibiting aromatic residues as potential stacking partners for the RNA bases. Anions located in various structures support the assignment of this RNA binding site. The proposed RNA binding region is hidden in the subunit interface of dimeric NusB proteins, such as NusB from M. tuberculosis, suggesting that such dimers have to undergo a considerable conformational change or dissociate for engagement with RNA. Therefore, in certain organisms, dimerization may be employed to package NusB in an inactive form until recruitment into antitermination complexes.