bluuebunny/crossref_metadata_2025_split huggingface dataset:
Iron metabolism in Parkinsonian syndromes
Authors: Berg Daniela , Hochstrasser Helmine 
Published: June 2006

AbstractGrowing evidence suggests an involvement of iron in the pathophysiology of neurodegenerative diseases. Several of the diseases are associated with parkinsonian syndromes, induced by degeneration of basal ganglia regions that contain the highest amount of iron within the brain. The group of neurodegenerative disorders associated with parkinsonian syndromes with increased brain iron content can be devided into two groups: (1) parkinsonian syndromes associated with brain iron accumulation, including Parkinson's disease, diffuse Lewy body disease, parkinsonian type of multiple system atrophy, progressive supranuclear palsy, corticobasal ganglionic degeneration, and Westphal variant of Huntington's disease; and (2) monogenetically caused disturbances of brain iron metabolism associated with parkinsonian syndromes, including aceruloplasminemia, hereditary ferritinopathies affecting the basal ganglia, and panthotenate kinase associated neurodegeneration type 2. Although it is still a matter of debate whether iron accumulation is a primary cause or secondary event in the first group, there is no doubt that iron‐induced oxidative stress contributes to neurodegeneration. Parallels concerning pathophysiological as well as clinical aspects can be drawn between disorders of both groups. Results from animal models and reduction of iron overload combined with at least partial relief of symptoms by application of iron chelators in patients of the second group give hope that targeting the iron overload might be one possibility to slow down the neurodegenerative cascade also in the first group of inevitably progressive neurodegenerative disorders. © 2006 Movement Disorder Society
Validity of the 30‐item geriatric depression scale in patients with Parkinson's disease
Authors: McDonald William M. , Holtzheimer Paul E. , Haber Michael , Vitek Jerrold L. , McWhorter Kimberly , DeLong Mahlon 
Published: June 2006

AbstractDepression in Parkinson's disease (dPD) is difficult to diagnose because depressive symptoms can overlap with symptoms of Parkinson's disease (PD). Subject‐rated scales such as the 30‐item Geriatric Depression Scale (GDS) may be useful in screening for dPD. There were 57 patients (33 men, 24 women; mean age, 58.6 years [SD ± 8.4]) enrolled in a study of pallidotomy for intractable PD who were evaluated for depression before and after surgery. Subjects were evaluated using the 17‐item Hamilton Depression Rating Scale (HDRS), Structured Clinical Interview for Diagnostic and Statistical Manual‐III (SCID), and the GDS. SCID was used to diagnose major depression with confirmation by an expert geropsychiatrist. Receiver‐operating curves (ROC) were used to identify cutoff points with maximal discriminant validity for diagnosing dPD. A total of 213 evaluation time points were included for the 52 patients with time points that included a valid SCID diagnosis, GDS, and HDRS. A ROC established points of maximum specificity/sensitivity for the GDS at a cutoff of 9/10 (sensitivity = 0.809, specificity = 0.837, positive predictive value [PPV] = 0.584, negative predictive value [NPV] = 0.939) and for the HDRS at a cutoff of 12/13 (sensitivity = 0.810, specificity = 0.821, PPV = 0.580, NPV = 0.934). The GDS was moderately correlated with the HDRS (Pearson's r = 0.54; P < 0.001). The GDS is useful in screening for dPD. A cutoff score of 9/10 has acceptable discriminant validity for dPD, and the GDS has a moderate correlation with the HDRS in PD patients. © 2005 Movement Disorder Society
A case of dysferlinopathy presenting choreic movements
Authors: Takahashi Toshiaki , Aoki Masashi , Imai Takashi , Yoshioka Masaru , Konno Hidehiko , Higano Shuichi , Onodera Yoshiaki , Saito Hiroshi , Kimura Itaru , Itoyama Yasuto 
Published: June 2006

AbstractMutations in the dysferlin gene cause limb–girdle muscular dystrophy type 2B (LGMD2B). The involvement of the central nervous system in dysferlinopathy has not been described. We describe the clinical features of a patient with LGMD2B associated with dysferlin mutations (homozygous G3370T) who presented progressive choreic movements. The patient had no evidence of other causes of chorea. It is suggested that the chorea may be associated with the altered expression of the brain isoform of dysferlin. © 2006 Movement Disorder Society
Practical and predictive bioinformatics methods for the identification of potentially cross‐reactive protein matches
Authors: Goodman Richard E. 
Published: June 2006

AbstractA bioinformatics comparison of proteins introduced into food crops through genetic engineering provides a mechanism to identify those proteins that may present an increased risk of allergic reactions for individuals with existing allergies. The goal is to identify proteins that are known to be allergens or are so similar to an allergen that they may induce allergic cross‐reactions. Three comparative approaches have traditionally been used, or considered for safety evaluations. One identifies any short (6–8) amino acid segment of the protein that exactly matches a known allergen sequence. The second is an overall primary sequence comparison using Basic Local Alignment Search Tool (BLAST) or FASTA to find matches of greater than 35% identity over 80 amino acids. The third is based on 3‐D prediction programs to identify 3‐D similarities that might predict potential cross‐reactivity. The utility of each of these approaches was debated in the bioinformatics workshop. The consensus agreement from the expert workshop participants was that the short‐segment match (e. g., 6–8 amino acids) provides an unacceptably high rate of false positive matches and an uncertain rate of true positive matches, and was not particularly useful for an allergenicity evaluation performed in the context of comprehensive safety evaluation. There was no consensus regarding the most appropriate bioinformatics method, an acceptable scoring criteria for triggering closer examination subsequent to a positive match, or an acceptable scoring mechanism for ranking the utility of the various 3‐D approaches that were discussed during the workshop. However, the general consensus was that the most practical approach at this time is to evaluate primary sequence identities to known allergens using either FASTA or BLAST. While there was good agreement that identities of greater than 35% over 80 or more amino acids (recommended by Codex in 2003) is quite conservative, the conclusion was that additional data or studies would be needed to justify changing this criterion as there is some evidence that some individuals sensitized to proteins in evolutionarily conserved protein families may experience cross‐reactions to proteins sharing approximately 40% identity.
Competition of nitroxyl contrast agents as an in vivo tissue redox probe: Comparison of pharmacokinetics by the bile flow monitoring (BFM) and blood circulating monitoring (BCM) methods using X‐band EPR and simulation of decay profiles
Authors: Okajo Aya , Matsumoto Ken‐ichiro , Mitchell James B. , Krishna Murali C. , Endo Kazutoyo 
Published: June 2006

AbstractNitroxyl radicals used as tissue redox‐sensitive contrast agents in electron paramagnetic resonance (EPR) and/or NMR imaging should satisfy the following two conditions: 1) the molecules disperse into tissues rapidly, and 2) paramagnetic loss occurs by simple reduction of the radical. The pharmacokinetic trends of several nitroxyl contrast agents were compared with the results obtained by bile flow monitoring (BFM) and blood circulation monitoring (BCM) methods using X‐band EPR. The nitroxyl radicals (TEMPO, TEMPONE (oxo‐TEMPO), and amino‐TEMPO) showed additional EPR signals in the bile that were attributed to metabolites formed during transport from blood to bile through the liver. However, the highly hydrophilic CAT‐1 (trimethylammonium‐TEMPO), which has low membrane permeability, showed minimal concentration in the bile. Probes that have carboxyl moiety, such as carboxy‐TEMPO and carboxy‐PROXYL, can be transported via anion transporter into hepatic cells. The EPR signal decay profiles of the nitroxyl radicals were simulated based on the experimental data. The simulation, which we previously applied to mouse blood, was modified to simultaneously fit the experimental results of BFM and BCM obtained with rats. The simulation data showed the simplicity/complexity of the pharmacokinetic mechanisms and that carbamoyl‐PROXYL and TEMPOL (hydroxy‐TEMPO) are suitable contrast agents for assessing tissue redox status. Magn Reson Med, 2006. Published 2006 Wiley‐Liss, Inc.
Fast automatic linear off‐resonance correction method for spiral imaging
Authors: Chen Weitian , Meyer Craig H. 
Published: June 2006

AbstractField inhomogeneity and susceptibility variations, coupled with a long readout, can result in image blurring in spiral imaging. Many correction methods based on a priori off‐resonance information, such as an acquired field map, have been proposed in the literature. Automatic off‐resonance correction methods are alternative approaches that estimate a field map from the image data themselves. In this paper we propose a fast automatic off‐resonance correction method that performs linear correction without acquiring a field map. The method requires only about two times the total computation time compared to image reconstruction by gridding. It can also be used in combination with a full field map automatic off‐resonance correction method to increase the extent of correction. The method is demonstrated by in vivo coronary artery imaging. Magn Reson Med, 2006. © 2006 Wiley‐Liss, Inc.
Developmental regulation of biglycan expression in muscle and tendon
Authors: Lechner Beatrice E. , Lim Jae H. , Mercado Mary Lynn , Fallon Justin R. 
Published: June 2006

AbstractBiglycan is an extracellular ligand for the dystrophin‐associated protein complex (DAPC) that is upregulated in both dystrophic and regenerating muscle. Biglycan also binds to collagen VI, mutations of which cause a congenital muscular dystrophy (Ullrich's; UCMD) that is also characterized by connective tissue abnormalities. The expression of biglycan in early development and postnatal ages has not been well characterized. Here we show that biglycan transcript levels peak at ∼21 weeks' gestation in human fetal muscle. Immunocytochemical analysis of developing mouse muscle shows that biglycan can be detected in muscle as early as embryonic day (E)16 and is most abundant between postnatal day (P)1 and P7. Biglycan is also highly expressed in developing tendon, with maximal levels observed at E16–18. This robust tendon expression is correlated with a sharp peak in biglycan transcript levels in the hindlimb. Finally, at E18 collagen VI colocalizes with biglycan in tendon. These results suggest that biglycan has a particularly important function during muscle and connective tissue development. Moreover, biglycan may play a role in the pathogenesis of collagen VI–associated congenital muscular dystrophies. Muscle Nerve, 2006
Approaches to the treatment of mitochondrial diseases
Authors: DiMauro Salvatore , Hirano Michio , Schon Eric A. 
Published: June 2006

AbstractTherapy for mitochondrial diseases is woefully inadequate. However, lack of a cure does not equate with lack of treatment. Palliative therapy is dictated by good medical practice and includes anticonvulsant medication, control of endocrine dysfunction, and surgical procedures. Removal of noxious metabolites is centered on combating lactic acidosis, but extends to other metabolites. Attempts to bypass blocks in the respiratory chain by administration of electron acceptors have not been successful, but this may be amenable to genetic engineering. Administration of metabolites and cofactors is the mainstay of real‐life therapy and is especially important in disorders due to primary deficiencies of specific compounds, such as carnitine or coenzyme Q10 (CoQ10). There is increasing interest in the administration of reactive oxygen radicals (ROS) scavengers, both in primary mitochondrial diseases and in neurodegenerative diseases. Gene therapy is a challenge because of polyplasmy and heteroplasmy, but novel experimental approaches are being pursued. One important strategy is to decrease the ratio of mutant to wild‐type mitochondrial genomes (“gene shifting”) by different means: (1) converting mutated mitochondrial DNA (mtDNA) genes into normal nuclear DNA genes (“allotopic expression”); (2) importing cognate genes from other species (“xenotopic expression”); (3) correcting mtDNA mutations by importing specific restriction endonucleases; (4) selecting for respiratory function; and (5) inducing muscle regeneration. Germline therapy raises ethical problems but is being considered for prevention of maternal transmission of mtDNA mutations. Preventive therapy through genetic counseling and prenatal diagnosis is becoming increasingly important for nuclear DNA–related disorders. Muscle Nerve, 2006
Impaired abdominal muscle contractility after high‐intensity exhaustive exercise assessed by magnetic stimulation
Authors: Verges Samuel , Schulz Christian , Perret Claudio , Spengler Christina M. 
Published: June 2006

AbstractHigh‐intensity exercise can induce diaphragm fatigue which can, in turn, limit exercise performance. We investigated whether expiratory muscles fatigue similarly during exhaustive exercise. Eleven healthy male volunteers cycled to exhaustion at 85% maximal power. Before, immediately after exercise, and after 30 and 60 min of recovery, the nerve roots supplying the abdominal muscles were stimulated magnetically at the T10 level in the prone position after full potentiation. Twitch gastric pressure (Pga,tw) was simultaneously recorded. After cycling, Pga,tw was significantly reduced compared to before exercise (40.2 ± 6.6 vs. 45.3 ± 7.5 cmH2O; P < 0.001), whereas after 30 and 60 min of recovery differences were no longer significant. The reduction in Pga,tw directly after exercise correlated neither with the fitness level nor with abdominal muscle work, respiratory sensations, or blood lactate concentration during exercise. These results indicate that the ventilatory requirements during intensive exercise can impair abdominal muscle contractility similar to diaphragmatic contractility. Thus, abdominal muscle fatigue may also contribute to exercise limitation, especially when expiratory resistance is increased as in patients with chronic obstructive pulmonary disease. Muscle Nerve, 2006
Survey of electrodiagnostic laboratories regarding hemorrhagic complications from needle electromyography
Authors: Gruis Kirsten L. , Little Ann A. , Zebarah Valerie A. , Albers James W. 
Published: June 2006

AbstractLittle is known about the complications of needle electromyography (EMG) performed on anticoagulated patients, and no guidelines exist regarding its performance. We conducted an anonymous survey of academic EMG laboratories in the U.S. to understand current practices and complications with regard to anticoagulated patients and those receiving antiplatelet medications. Forty‐seven (78%) of 60 EMG laboratories responded to the survey. Four laboratories (9%) reported at least one hemorrhagic complication requiring medical or surgical intervention in an anticoagulated patient, whereas none reported a hemorrhagic complication in patients receiving antiplatelet medications. Ten (21%) reported willingness to evaluate cranial, paraspinal, and all limb muscles in anticoagulated patients. This survey suggests that hemorrhagic complications from needle EMG of anticoagulated patients are rare. It also suggests that needle EMG of patients receiving antiplatelet therapy is not associated with increased reports of hemorrhagic complications. Muscle Nerve, 2006
Peak power of muscles injured by lengthening contractions
Authors: Widrick Jeffrey J. , Barker Tyler 
Published: June 2006

AbstractExcessive or extreme lengthening contractions have a well‐characterized depressive effect on skeletal muscle isometric force. In addition to producing force, active muscles must often shorten in order to meet the power requirements of locomotion and other physical activities. However, the impact of lengthening contractions on muscle power is poorly understood. We evaluated the effect of 20 isometric contractions or 20 lengthening contractions (20% strain at 1.5 fiber lengths/s) on the force–velocity–power relationships of mouse soleus muscles in vitro at 35°C. Pre‐ and posttreatment data were obtained as the muscles shortened through their optimal length (Lo). The isometric treatment did not alter Lo, the curvature of the force–velocity relationship (a/Po), or soleus maximal shortening velocity (Vmax), whereas peak force (Po) displayed a slow, time‐dependent decline of 10% across the experiments. Following the lengthening treatment, Lo increased by 6%, a/Po increased by 22%, and Vmax and Po fell by 24% and 26%, respectively. Under optimal conditions for producing power, muscles damaged by lengthening contractions attained 22% less force and shortened 20% more slowly than before damage. Consequently, soleus peak power fell 37% after lengthening, a 2.5‐fold greater decline than noted for the isometric treatment. Under the conditions studied here, the excessive power loss following lengthening contractions was due to force and velocity deficits of approximately equal relative magnitude. Because power represents the ability of the muscle to perform work, reductions in both force and shortening velocity should be considered when evaluating and treating lengthening‐induced skeletal muscle injuries. Muscle Nerve, 2006
Relationship between abdominal and pelvic floor muscle activation and intravaginal pressure during pelvic floor muscle contractions in healthy continent women
Authors: Madill Stéphanie J. , McLean Linda 
Published: June 2006

AbstractAimActivation of the abdominal muscles might contribute to the generation of a strong pelvic floor muscle contraction, and consequently may contribute to the continence mechanism in women. The purpose of this study was to determine the abdominal muscle activation levels and the patterns of muscle activity associated with voluntary pelvic floor muscle (PFM) contractions in urinary continent women.MethodsFifteen healthy continent women participated. They performed three maximal contractions of each of the four abdominal muscles and of their PFMs while in supine. Abdominal and PFM activity was recorded using electromyography (EMG), and intravaginal pressure was recorded using a custom modified Femiscan™ probe.ResultsDuring voluntary maximal PFM contractions, rectus abdominus was activated to 9.61 (±7.42)% maximal voluntary electrical activity (MVE), transversus abdominus was activated to 224.30(±47.4)% MVE, the external obliques were activated to 18.72(±13.33)% MVE, and the internal obliques were activated to 81.47(±63.57)% MVE. A clear pattern of activation emerged, whereby the transversus abdominus, internal oblique, and rectus abdominus muscles worked with the PFM in the initial generation of maximal intravaginal pressure. PFM activity predominated in the initial rise in lower vaginal pressure, with later increases in pressure (up to 70% maximum pressure) being associated with the combined activation of the PFM, rectus abdominus, internal obliques, and transverses abdominus. These abdominal muscles were the primary source of intravaginal pressure increases in the latter 30% of the task, whereas there was little increase in PFM activation from this point on. The external oblique muscles showed no clear pattern of activity, but worked at approximately 20% MVE throughout the PFM contractions, suggesting that their role may be predominantly in postural setting prior to the initiation of intravaginal pressure increases.ConclusionsDefined patterns of abdominal muscle activity were found in response to voluntary PFM contractions in healthy continent women. Neurourol. Urodynam. 25:722–730, 2006. © 2006 Wiley‐Liss, Inc.
Development of an HPLC‐PAD‐MS assay for the identification and quantification of major phenolic edelweiss (Leontopodium alpium Cass.) constituents
Authors: Schwaiger Stefan , Seger Christoph , Wiesbauer Bettina , Schneider Peter , Ellmerer Ernst P. , Sturm Sonja , Stuppner Hermann 
Published: June 2006

AbstractThe analytical assessment of edelweiss (Leontopodium alpinum) herb extracts, used in traditional alpine medicine, has resulted in the development of a HPLC‐PAD‐MS method that allows baseline separation of almost all constituents. Peak assignment of 14 analytes was achieved by comparison of retention times, UV and mass spectra with those of reference compounds either commercially available (luteolin, apigenin and chlorogenic acid) or isolated from edelweiss plants by column chromatography. Ten of the isolated analytes were identified as the known natural products: quercetin‐3‐O‐β‐d‐glucoside, luteolin‐7‐O‐β‐d‐glucoside, luteolin‐3′‐O‐β‐d‐glucoside, luteolin‐4′‐O‐β‐d‐glucoside, apigenin‐7‐O‐β‐d‐glucoside, 6‐hydroxy‐luteolin‐7‐O‐β‐d‐glucoside, luteolin‐7,4′‐di‐O‐β‐d‐glucoside, chrysoeriol‐7‐O‐β‐d‐glucoside, leontopodic acid and 3,5‐dicaffeolyquinic acid. One analyte, 3,4,5‐tri‐(E)‐caffeoly‐d‐glucaric acid proved to be a new natural product and was named leontopodic acid B. Structure elucidation was carried out by means of MS and NMR spectroscopy in all cases. The aerial plant parts of L. alpinum (capitula, inflorescence leaves, stems, stem leaves and leaves of the basal rosette) showed variable amounts of the above‐mentioned constituents, although qualitative differences were not observable. Copyright © 2006 John Wiley & Sons, Ltd.
Some safety aspects on the design of sparger systems for the oxidation of organic liquids
Authors: Weber Manfred 
Published: June 2006

AbstractThis article summarizes all important parameters for a proper and safe design of the sparger system for large bubble columns, used for the oxidation of organic liquids with air or oxygen enriched air. As an example, this is done for the oxidation of cumene (isopropylbenzene), which is worldwide used in phenol processes. Based on data in the literature and newly measured flammability limits at 100° C and 5 barg, the potential hazards from uncontrolled gas reactions can be avoided, ultimately avoiding an explosion in the sparger system. For normal operation, a minimum flow of gas is necessary to ensure a uniform flow through all outlets, which are typically holes with a diameter of several millimeters. All holes should be directed towards the vessel bottom to self‐drain the sparger. For start‐up, a previous inerting of the sparger with nitrogen is preferable. Nevertheless, a certain amount of organic liquid may still be in the sparger system prior to start up. Therefore, the compressed air/oxygen should have a temperature below the Oxidation Initiation Temperature to avoid any initiation of a liquid phase oxidation within the sparger. In addition, the pressure in the sparger system should be high enough to keep the gas phase concentration of the organic liquid below the Lower Flammability Limit. © 2006 American Institute of Chemical Engineers Process Saf Prog, 2006