bluuebunny/crossref_metadata_2025_split huggingface dataset:
DJ‐1 colocalizes with tau inclusions: A link between parkinsonism and dementia
Authors: Rizzu Patrizia , Hinkle David A. , Zhukareva Victoria , Bonifati Vincenzo , Severijnen Lies‐Anne , Martinez Daniel , Ravid Rivka , Kamphorst Wouter , Eberwine James H. , Lee Virginia M.‐Y. , Trojanowski John Q. , Heutink Peter 
Published: December 2003

AbstractTwo novel mutations recently have been identified in the DJ‐1 gene that cause a new form of autosomal recessive, early‐onset parkinsonism. Because the pathological role of this protein is unknown, we examined the issue here and report the colocalization of DJ‐1 protein within a subset of pathological tau inclusions in a diverse group of neurodegenerative disorders known as tauopathies. Our study extends the view that different neurodegenerative diseases may have similar pathological mechanisms, and that these processes likely include DJ‐1.
Pathomechanisms of neurodegeneration in glutaryl‐CoA dehydrogenase deficiency
Authors: Kölker Stefan , Koeller David M. , Okun Jürgen G. , Hoffmann Georg F. 
Published: December 2003

AbstractGlutaryl‐CoA dehydrogenase deficiency is an inherited organic aciduria with predominantly neurological presentation. Biochemically, it is characterized by an accumulation and enhanced urinary excretion of two key organic acids, glutaric acid and 3‐hydroxyglutaric acid. If untreated, acute striatal degeneration is often precipitated by febrile illnesses during a vulnerable period of brain development in infancy or early childhood, resulting in a dystonic dyskinetic movement disorder. The mechanism underlying these acute encephalopathic crises has been partially elucidated using in vitro and in vivo models. 3‐Hydroxyglutaric and glutaric acids share structural similarities with the main excitatory amino acid glutamate and are considered to play an important role in the pathophysiology of this disease. 3‐Hydroxyglutaric acid induces excitotoxic cell damage specifically via activation of N‐methyl‐D‐aspartate receptors. Furthermore, glutaric and 3‐hydroxyglutaric acids indirectly modulate glutamatergic and GABAergic neurotransmission, resulting in an imbalance of excitatory and inhibitory neurotransmission. It also has been suggested that secondary amplification loops potentiate the neurotoxic properties of these organic acids. Probable mechanisms for this effect include cytokine‐stimulated nitric oxide production, a decrease in energy metabolism, and reduction of cellular creatine phosphate levels. Finally, maturation‐dependent changes in the expression of neuronal glutamate receptors may affect the vulnerability to 3‐hydroxyglutaric and glutaric acid toxicity.
Identification of amino‐terminally cleaved tau fragments that distinguish progressive supranuclear palsy from corticobasal degeneration
Authors: Arai Tetsuaki , Ikeda Kenji , Akiyama Haruhiko , Nonaka Takashi , Hasegawa Masato , Ishiguro Koichi , Iritani Shuji , Tsuchiya Kuniaki , Iseki Eizo , Yagishita Saburo , Oda Tatsuro , Mochizuki Akihide 
Published: December 2003

AbstractProgressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are neurodegenerative diseases that are characterized by intracytoplasmic aggregates of hyperphosphorylated tau with four microtubule‐binding repeats. Although PSP and CBD have distinctive pathological features, no biochemical difference in aggregated tau has been identified. In this study, we examined the brains of eight patients with PSP, six patients with CBD, and one atypical case with pathological features of both CBD and PSP. On immunoblots of sarkosyl‐insoluble brain extracts, a 33kDa band predominated in the low molecular weight tau fragments in PSP, whereas two closely related bands of approximately 37kDa predominated in CBD. Immunoblots of the atypical case showed both the 33kDa band and the 37kDa doublet. Protein sequencing and immunochemical analyses showed that the 33kDa band and the 37kDa doublet consisted of the carboxyl half of tau with different amino termini. These results suggest that, despite the identical composition of tau isoforms, different proteolytic processing of abnormal tau takes place in these two diseases. Such a biochemical divergence may be related to the neuropathological features of these diseases.
Spinocerebellar ataxia with sensory neuropathy (SCA25) maps to chromosome 2p
Authors: Stevanin Giovanni , Bouslam Naima , Thobois Stéphane , Azzedine Hamid , Ravaux Lucas , Boland Anne , Schalling Martin , Broussolle Emmanuel , Dürr Alexandra , Brice Alexis 
Published: December 2003

AbstractAutosomal dominant cerebellar ataxias constitute one of the most clinically, neuropathologically, and genetically heterogeneous groups of neurodegenerative disorders. Approximately 50 to 80% of the families carry mutations in genes known to be implicated in spinocerebellar ataxias (SCAs). Numerous loci (SCAn) also have been mapped, often in single families, but the responsible genes have not yet been identified. This suggests further genetic heterogeneity. We have ascertained 18 subjects from a large French family in which cerebellar ataxia and prominent sensory neuropathy segregated as a dominant trait. Intrafamilial variability was high regarding age at onset (17 months to 39 years), severity, and the clinical picture that ranged from pure sensory neuropathy with little cerebellar involvement to a Friedreich's ataxia‐like phenotype. After excluding known genes/loci responsible for SCA and hereditary sensory neuropathies, we detected linkage with chromosome 2p markers in a genomewide screen. We designated this new locus SCA25 after testing of 16 additional markers. Maximum two‐point logarithm of odds scores of 3.15 and 3.10 were obtained at D2S2378 and D2S2734, respectively. Haplotype analysis defined a critical 12.6cM region of 15Mb between D2S2174 and D2S2736. No linkage to this locus was found in four other families. This interval contains several genes that could be responsible for the disease. One of these genes, CRIPT, encodes a postsynaptic protein, but no mutations were found by direct sequencing, excluding its responsibility in the disease. CAG repeat expansions often are involved in SCA pathogenesis, but no pathological expansions were found at the protein or at the DNA level using the 1C2 antibody and the repeat expansion detection method, respectively. The gene responsible for SCA25 remains to be identified.
Chromosomal translocation t(18;21)(q23;q22.1) indicates novel susceptibility loci for frontotemporal dementia with ALS
Authors: Prudlo Johannes , Alber Burkhard , Kalscheuer Vera M. , Roemer Klaus , Martin Thomas , Dullinger Joern , Sittinger Helmut , Niemann Stephan , Heutink Peter , Ludolph Albert C. , Ropers Hilger H. , Zang Klaus , Meyer Thomas 
Published: December 2003

AbstractA chromosomal translocation t(18;21)(q23;q22) is reported in a patient with frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). We exclude the physical involvement and silencing of the ALS‐linked gene for copper/zinc superoxide dismutase (SOD1) on chromosome 21q22.1. The breakpoints are assigned to sequences flanked by the markers ATA1H06, D18S462, D21S1915, and D21S1898. These critical regions may contain susceptibility loci for FTD associated with ALS.
The effect of spinal cord stimulation in patients with chronic reflex sympathetic dystrophy: Two years' follow‐up of the randomized controlled trial
Authors: Kemler Marius A. , De Vet Henrica C. W. , Barendse Gerard A. M. , Van Den Wildenberg Frans A. J. M. , Van Kleef Maarten 
Published: December 2003

AbstractChronic reflex sympathetic dystrophy is a painful, disabling disorder for which no treatment with proven effect is available. We performed a randomized trial in a 2 to 1 ratio of patients, in which 36 patients were treated with spinal cord stimulation and physical therapy (SCS+PT), and 18 patients received solely PT. Twenty‐four SCS+PT patients were given a permanent spinal cord stimulation system after successful test stimulation; the remaining 12 patients received no permanent system. We assessed pain intensity, global perceived effect, functional status, and health‐related quality of life. Patients were examined before randomization, before implantation, and also at 1, 3, 6, 12, and 24 months thereafter. At 2 years, three patients were excluded from the analysis. The intention‐to‐treat analysis showed improvements in the SCS+PT group concerning pain intensity (−2.1 vs 0.0cm; p < 0.001) and global perceived effect (43% vs 6% “much improved”; p = 0.001). There was no clinically important improvement of functional status. Health‐related quality of life improved only in the group receiving spinal cord stimulation. After careful selection and successful test stimulation, spinal cord stimulation results in a long‐term pain reduction and health‐related quality of life improvement in chronic reflex sympathetic dystrophy.
Phosphorus Nuclear Magnetic Resonance Spectroscopy
Authors: Jones Richard A. Y. , Katritzky A. R. 
Published: December 2003

AbstractNuclear magnetic resonance of the 31P nucleus is suitable for the structural elucidation of phosphorus compounds, also for analytical and kinetic studies. In this paper, an attempt has been made towards the presentation of a comprehensive review of all 31P resonances so far determined.
Mixed‐Phase Pigments with Rutile Structures
Authors: Hund F. 
Published: December 2003

AbstractUntil the present time, new color pigments have been developed by isotypic and homotypic mixed‐phase formation. This paper deals primarily with heterotypic mixed‐phase formation for the preparation of new colored and white pigments with the rutile structure. Dioxides and difluorides crystallizing in the rutile lattice‐form were chosen as host‐lattices for the mixed‐phase formation. Hosts of the general formula AB2 can accommodate, as guests, in the form of solid solutions, oxides and/or fluorides with different structures. Characteristically, the mean statistical cation‐radius lies between 0.55 and 0.98 Å, with single radii between 0.46 and 0.98 Å. Relative quantities are so chosen that the cation to anion ratio of 1:2 prescribed by the host lattice, as well as electroneutrality, is maintained.
The Application of Mass Spectrometry in Organic Chemistry: Determination of the Structure of Natural Products
Authors: Biemann K. 
Published: December 2003

AbstractMass spectrometry is a very useful method for the determination of the structure of organic compounds although the technique has not yet been widely employed in this field. Of particular interest is the high sensitivity of the instrument, making possible the use of very small amounts of sample, and the information obtainable regarding the size of the molecule and position of the groups therein. A certain volatility is required and the choice of a suitable derivative is, therefore, sometimes of importance. The fragmentations of the molecules in the ion source may be considered as reactions of “carbonium ions without solvent.” The application of the method in the determination of the structure of some natural products is outlined, using lipids, amino acids, peptides and alkaloids as examples.
Inverse Polarography and Voltammetry: New Methods for Trace Analysis
Authors: Neeb Rolf 
Published: December 2003

AbstractInverse polarographic and voltammetric methods are surveyed. The electrochemical determination procedure is carried out by current reversal after electrolytic concentration on a working electrode. Analyses in the nanogram (ng) region (10−9 g.) are possible because the sensitivity is increased by a factor of 100 to 1000 in comparison with the usual polarographic and voltammetric methods. These methods are, therefore, of particular interestin trace analysis.
The Mechanism of Bimolecular β‐Elimination Reactions
Authors: Bunnett J. F. 
Published: December 2003

AbstractIn bimolecular β‐elimination (E 2 mechanism), several bonds are ruptured or formed in one concerted reaction step. However, the various aspects of bond‐making or ‐breaking need not be completely synchronous. In the E 2 transition state for elimination HX, rupture of the CX bond may be more advanced than that of the CH linkage, or vice versa. Factors influencing the relative extents of rupture of these two bonds at the transition state are discussed, and the consequences of non‐synchroneity in one sense or the other are developed from theory and illustrated from experiment. This treatment provides an understanding of the Hofmann‐vs.‐Saytzeff orientation problem.
Recent Developments in Non‐Mercurial Diuretics
Authors: Schlittler E. , Destevens G. , Werner L. 
Published: December 2003

AbstractMercurial diuretics today are still the drugs of choice when a prompt and full diuretic response is needed. However, most mercurial diuretics are effective only when given parenterally. This factor, plus the toxicity usually associated with organic mercurials; has prompted the search for non‐toxic, orally active, non‐mercurial diuretic drugs. The most important group of organic compounds found to date which fulfills these conditions is the sulfonamides. Among these, derivatives of chlorothiazide and hydrochlorothiazide are most effective. In the following report these compounds are described in detail. Aldosterone antagonists and blockers of aldosterone excretion play only a minor role.
Thin‐Layer Chromatography of Nucleotides
Authors: Randerath K. 
Published: December 2003

AbstractThin‐layer chromatography of nucleotides on DEAE‐ and ECTEOLA‐cellulose is superior to the methods of seperation in use up to the present. Using dilute hydrochloric acid, and without a gradient elution technique, it is possinle to acheive a group seperation of monodi‐, and tri‐phosphates as well as subfractionation within these groups depending on the basicity of the nucleobases. Thin‐layer chromatography is appreciably more sensitive than paper chromatography; 5×10−4 mole adenine neuclotides can be still be detected. The total quantity of material that can be chromatographed without tailing is greater in this procedure than that with paper chromatography; a mixture consisting of 1 μmole ATP and ADP per starting spot can be resolved with ease . Hence the procedure can also be applied for micro‐scale preparative seperations . A mixtures of 10−2 μmole ADP and ATP, respectively, can be seperated completely in four to ten minutes.
The Synthesis of Nucleosides
Authors: Ulbricht T. L. V. 
Published: December 2003

AbstractThe most important developments in nucleoside synthesis in the last few years have been the application of the mercury salt procedure to both purines and pyrimidines, and the isolation of relatively stable, crystalline 2‐deoxyribofurnosyl halides; as a result, almost any nucleoside may now be synthesized. Earlier syntheses, based on older methods or on the conversion of one nucleoside to another, played an essential part in establishing the structure of the nucleosides (and hence of the nucleic acids).
β‐Lactams, Their Polymerization and Use as Raw Materials for Fibers
Authors: Graf R. , Lohaus G. , Börner K. , Schmidt Erwin , Bestian H. 
Published: December 2003

Abstractβ‐Lactams can be prepared by cyclization of β‐aimno acid esters. Recently they have become available also from olefins by addition of N‐carbonysulfamyl chloride (isocyanatosulfonyl chloride) and from aldehydes by reaction with N‐carbonylsulfamyl chloride and ketene. Condensative or anionic polymerization results in polyamides the chains of which contain many more amide groups than the chains of polyamides of the nylon‐6 type. Hence the new polymers resemble silk moreclosely. Fibers and films can be prepared from solution.
The Isomerization of Aziridine Derivatives
Authors: Heine H. W. 
Published: December 2003

AbstractDerivatives of aziridine or ethylenimine are isomerized by acids, nucleophilic reagents, or pyrolysis. All of the isomerizations reported so far are ring‐opening reactions, predominantly involving cleavage of the carbon‐nitrogen bond of the ring, although a few isomerizations are known in which the carbon‐carbon bond of the ring is broken. Isomerization of appropriate aziridine derivatives provides useful synthetic pathways leading to heterocyclic systems such as oxazolines, imidazolines (and related systems), imidazolidinoes, thiazolines, pyrazolines, and triazolines. Other isomerizations form N‐unsaturated amides, anils, and isocyanates.
The Preparation of Carbodiimides from Isocyantes
Authors: Neumann W. , Fisher Peter 
Published: December 2003

AbstractCarbodiimides are formed with simultaneous evolution of CO2 by the reaction of basic catalyst with sterically hindered isocynates. Another generally applicable synthesis of carbodiimides involves intermediate formation of cyclic adducts of isocyanates and carbodiimides, which, upon cleavage, exchange the isocyanate and carbodiimide functions. Both processes are suitable for the preparation of polycarboiimides. Several commercial applications of mono‐ and polycarbodiimides are pointed out.
Syntheses Using the Michael Adddition of Phridinium Salts
Authors: Kröhnke F. , Zecher W. , Curtze J. , Drechsler D. , Pfleghar K. , Schnalke K. E. , Weis W. 
Published: December 2003

AbstractBy Michael addition of the active methylene groups in pyridinium salts onto suitable acceptor compounds, α‐pyridones, substituted pyridines, particularly pyridinecarboxylic acids and pyridylpyridines, including the minor alkaloid of tobacco nicotelline and annelized pyridines, can be prepared by a simple procedure and generally in good yields. From the Michael adducts, polycyclic aromatic hydrocarbons, e.g. substituted fluoranthenes and “bisfluoranthenes” can be prepared; internal Michael addition leads to pyrrolinopyrdinium salts. For example, Michael addition of pyridinium salts onto quinones gives phenacyl substituted quinones, from which benzofurans and cinnolines can be readily obtained.
Heterocyclic Azo Dyes by Oxidative Coupling
Authors: Hünig S. , Balli H. , Breither E. , Brühne F. , Geiger H. , Grigat E. , Müller F. , Quast H. 
Published: December 2003

AbstractNew findings in the field of oxidative coupling reactions are reported. These involve: extension of this type of reaction to amidrazone system displaying (vinylogous) tautomerism and to unsymmetrical diarylhydrazines; elucidation of the coupling mechanism for these arylhydrazines and for the amidrazone systems previously described; the properties of some dyes, complexed with heavy metals, and the synthesis of tetraazapentamethine and penta‐azapentamethine dyes.
β‐Chloroethylphosphonic Dichloride: Its Synthesis and Use
Authors: Rochlitz F. , Vilcsek H. 
Published: December 2003

AbstractEthylene, phosphorus trichloride, and oxygen react together to give β‐chloroethylphosphonic dichloride in good yields. Being a bifunctional acid chloride, this compound undergoes polycondensation reactions with other, at least bifunctional, compounds affording polyesters, polyamides, epoxy resins, etc. β‐Chloroethylphosphonic dichloride also may be converted into vinylphosphonic dichloride, thus serving as a starting material for the preparation of vinylphosphonic acid and its derivatives. These undergo both homopolymerization and copolymerization with compounds containing olefinic double bonds. The products are used in the field of synthetic resins, for dressing textiles, and as protective surface coatings.
Polycondensation Dyes. A New Principle for the Preparation of Wash‐Fast Cotton Dyeings
Authors: Schimmelschmidt K. , Hoffmann Hermann , Baier E. 
Published: December 2003

AbstractDyes which contain only thiosulfate residues as water‐solubilizing groups are readily polycondensed on cellulose under mild conditions in the presence of condensing agents such as sodium sulfide to give insoluble, high molecular‐weight dyes. This type of fixation leads to wash‐fast dyeings and, in contrast to reactive dyes, involves no reaction with the fiber. The preparation and application of the dyes are described.
Conductivity and Surface Chemistry of Crystals
Authors: Schwab G.‐M. 
Published: December 2003

AbstractDoping of metals and semiconductors can change both their electrical conductivity and catalytic activity. The change in conductivity gives information about the number of occupied and vacant electron levels. If the rate‐determining step of a catalytic reaction involving a doped solid can be identified, then a relationship between the conductivity and chemical behavior of the solid may be established.
The Silicate Bond from the Standpoint of Electronic Theory
Authors: Noll W. 
Published: December 2003

AbstractStarting from a consideration of the “undisturbed” SiOSi bond in the modification of silicic acid, the types of bonds present in silicates with polymeric SiOSi groups are discussed, taking into consideration the inductive effect of the metal cations. Experimental data on the influence of various substituents in organopolysiloxanes form the basis for this discussion. The bonds in silicates differ by direction and character and are determined by the relative ability of the metal atoms to act as donors and by the degree of condensation of the SiOSi bond. It is shown that on this basis, it is possible to explain, for example, why silicates differ in their atomic distances, susceptibility to cleavage by acids, stability relationships, and other properties.
Thermal Rearrangements
Authors: von E. Doering W. , Roth W. R. 
Published: December 2003

AbstractThe effect of conjugation with double bonds and (in cyclopropane) the effect of ring strain on the cleavage of carbon‐carbon bonds during thermal rearrangements is discussed. In particular, the Cope rearrangement and the geometry of its transition state are described. The structurally degenerate Cope rearrangement of bicyclo[5. 1. 0]octa‐2,5‐diene is discussed, and this compound is used to illustrate the concept of “fluctional structure”, which finds its extreme expression in the hypothetical compound, tricyclo[3. 3. 2. 04,6]‐deca‐2,7,9‐triene.
Fulvenes as Isomers of Benzenoid Compounds
Authors: Hafner K. , Häfner K. H. , König C. , Kreuder M. , Ploss G. , Schulz G. , Sturm E. , Vöpel K. H. 
Published: December 2003

AbstractWith regard to the nature of their bonding and reactivity, fulvenes occupy a position intermediate between their benzenoid isomers and the olefins. The chemical and physical behavior of the fulvenes is determined either by the diene character of the cross‐conjugated system or by the cylic conjugation in the five‐membered ring, depending on the type of substituent at the exocyclic carbon atom. In addtion to several new substitution reactions, a description is given of the syntheses and reactions of 6‐amino‐ and 6‐hydroxyfulvenes, isomeric with anilines and phenols, the derivatives of which can be used for the preparation of new types of nonbenzenoid cyclic conjugated systems such as carbocyclic and heterocyclic azulenes, pseudoazulenes, thiepines, dihydropyridazines, and s‐indacene.
Syntheses Using Pyridinium Salts
Authors: Kröhnke Fritz 
Published: December 2003

AbstractThis review discusses syntheses leading to a new type of N‐vinylpyridinium salts, to novel anionocyanines, to aminophenazines and alloxazines, as well as to (aza‐) dehydroquinolizinium, acridizinium, and morphanthridizinium salts. In addition, Mannich reactions and aminoalkylations at active methylene groups of cycloimmonium salts, and their reactions with amines are treated. The possibilities offered by the “Ortoleva‐King” reaction are demonstrated using numerous examples, such as a new route leading to the yohimbine skeleton.