bluuebunny/crossref_metadata_2025_split huggingface dataset:
Novel heterozygous missense mutation in the platelet glycoprotein Ibβ gene associated with isolated giant platelet disorder
Authors: Kunishima Shinji , Naoe Tomoki , Kamiya Tadashi , Saito Hidehiko 
Published: September 2002

AbstractThe glycoprotein (GP) Ib/IX/V complex plays an important role in primary hemostasis, serving as the platelet receptor for von Willebrand factor (vWF). Recent studies have shown that the phenotype caused by mutations in the subunits of the GPIb/IX complex spans a wide spectrum; from the normal phenotype, to isolated giant platelet disorders (GPD), and to the full‐blown bleeding disorder, the Bernard‐Soulier syndrome (BSS). We characterize here a novel missense mutation of the GPIbβ gene associated with isolated GPD. In the patient's platelets, the expression level of the GPIb/IX complex was moderately reduced compared with that of the GPIIb/IIIa complex, whereas the latter was expressed at higher levels than in a normal control. Immunoblot analysis showed normal electrophoretic mobility of GPIbα, GPIbβ, and GPIX. However, the amount of GPIbβ was approximately 66% of the normal value. DNA sequencing analysis revealed a novel heterozygous missense mutation in the GPIbβ gene that converts Arg (CGC) to Cys (TGC) at residue 17. Transient transfection studies demonstrated that mutant GPIbβ protein was not detected in transfected 293T cells. These findings indicated that null expression of the abnormal GPIbβ causes decreased expression of the complex and results in the GPD phenotype in the patient, and suggested that homozygosity of the mutation may lead to a BSS phenotype in vivo. Am. J. Hematol. 68:249–255, 2001. © 2001 Wiley‐Liss, Inc.
Localized herpes simplex lymphadenitis mimicking large‐cell (Richter's) transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma
Authors: Joseph Lija , Scott Margie A. , Schichman Steven A. , Zent Clive S. 
Published: September 2002

AbstractWe report a patient with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) who presented with rapid enlargement of a cervical lymph node due to localized herpes simplex lymphadenitis, which was clinically indistinguishable from large cell (Richter's) transformation. The diagnosis was made by excisional lymph node biopsy, which demonstrated CLL/SLL and zonal necrosis due to herpes simplex infection. The herpetic zone was surrounded by a brisk proliferation of immunoblasts. This case demonstrates the need for excisional biopsy and histologic examination of rapidly enlarging nodes in patients with CLL/SLL. The diagnosis of herpes simplex lymphadenitis in patients with CLL/SLL is especially important because, unlike large cell transformation, the infection usually responds well to treatment. Am. J. Hematol. 68:287–291, 2001. Published 2001 Wiley‐Liss, Inc.
Lack of allelic exclusion and isotype switching in B cell chronic lymphocytic leukemia
Authors: Katayama Yuta , Sakai Akira , Katsutani Shinya , Takimoto Yasuo , Kimura Akiro 
Published: September 2002

AbstractTwo rearranged bands of the IgH gene were detected in a case with B cell chronic lymphocytic leukemia (B‐CLL). The expressed VH gene was only VH1 with no somatic mutations in IgM and IgD. The expressions of Cμ, Cδ, and Cγ were detected by reverse transcription of RNA followed by the polymerase chain reaction (RT‐PCR). Sequence analysis of the CDR3 regions of each PCR reaction product showed that the sequence of one rearranged allele was identical to those of the expressed VH1 gene, Cμ and Cδ, and the sequence of another rearranged allele was identical to that of Cγ. However, none of the expressed VH genes was detected in IgG. These findings suggest that this is a case of B‐CLL lacking allelic exclusion and undergoing a class switch of one allele with the incomplete expression of the VH gene. Am. J. Hematol. 68:295–297, 2001. © 2001 Wiley‐Liss, Inc.
Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy
Authors: Hughes Richard , Bensa Siân , Willison Hugh , Van Den Bergh Peter , Comi Giancarlo , Illa Isabel , Nobile‐Orazio Eduardo , Van Doorn Pieter , Dalakas Marinos , Bojar Martin , Swan Anthony ,   Inflammatory Neuropathy Cause and Treatment (INCAT) Group
Published: September 2002

AbstractThis multicenter, randomized, double‐blind, crossover trial compared a 6 week course of oral prednisolone tapering from 60 mg to 10 mg daily with intravenous immunoglobulin (IVIg) 2.0 g/kg given over 1 to 2 days for treating chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Twenty‐four of the thirty‐two randomized patients completed both treatment periods. Both treatments produced significant improvements in the primary outcome measure, change in an 11‐point disability scale 2 weeks after randomization. There was slightly, but not significantly, more improvement after IVIg than with prednisolone, the mean difference between the groups in change in disability grade being 0.16 (95% CI = –0.35 to 0.66). There were also slightly, but not significantly, greater improvements favoring IVIg in the secondary outcome measures: time to walk 10 meters after 2 weeks and improvement in disability grade after 6 weeks. Results may have been biased against IVIg by the 8 patients who did not complete the second arm of the trial. A serious adverse event (psychosis) attributable to treatment occurred in 1 patient while on prednisolone and in none with IVIg.
Adult neuronal ceroid lipofuscinosis with palmitoyl‐protein thioesterase deficiency: First adult‐onset patients of a childhood disease
Authors: Van Diggelen Otto P. , Thobois Stephane , Tilikete Caroline , Zabot Marie‐Thérèse , Keulemans Joke L. M. , Van Bunderen Patrick A. , Taschner Peter E. M. , Losekoot Monique , Voznyi Yakov V. 
Published: September 2002

AbstractThe fluorogenic enzyme assay for palmitoyl‐protein thioesterase (PPT) has greatly facilitated the diagnosis of infantile neuronal ceroid lipofuscinosis (Santavuori‐Haltia disease) and the search for possible new variants with atypical clinical presentation. Here, we present the first cases of adult neuronal ceroid lipofuscinosis with onset in the fourth decade of life due to a profound deficiency of PPT. The causative mutations in the CLN1 gene were the known, deleterious mutation R151X and the novel missense mutation G108R. Patients presented at onset (31 and 38 years), with psychiatric symptoms only. At present (ages 56 and 54 years), visual, verbal, and cognitive losses have progressed and both patients have cerebellar ataxia and cannot walk without support.
Effect of the oxidation conditions on the maghemites produced by laser pyrolysis
Authors: Veintemillas‐Verdaguer S. , Morales M. P. , Serna C. J. 
Published: September 2002

AbstractPure γ − Fe2O3 particles were continuously prepared by a continuous wave CO2 laser‐induced pyrolysis of iron pentacarbonyl vapour in an oxidizing atmosphere. Two different oxidation procedures were investigated: (1) air–argon mixtures were employed to fill the apparatus; (2) air–ethylene mixtures were employed as carrier gas. The powders were identified by X‐ray diffraction. The grain size was measured using transmission electron microscopy and the degree of order was estimated by IR spectroscopy. The products obtained have grain sizes in the range 4 to 17 nm and different degrees of structural order depending on the synthesis conditions. The productivities attained are typically 0.05 gh−1. Finally, the magnetic properties of the maghemite powders are discussed. It seems that, for very small magnetic particles, the actual differences in the sizes of the particles are less important than differences in crystallinity with regard to the magnetic properties of the samples. Copyright © 2001 John Wiley & Sons, Ltd.
Thin films of nanomaterials made using intense cluster beams
Authors: Eastham D. A. , Kirkman I. W. , Denby P. M. 
Published: September 2002

AbstractThin films of material containing embedded nanocrystals in the size range from 1.5 to 6 nm have been manufactured using intense cluster beams. A description of the equipment is given, as well as its measured performance. Measurements of the morphology of the material have been made directly using transmission electron microscopy and, indirectly, with SQUID magnetometry. These measurements are consistent with the hypothesis that the material consists of approximately spherical nanocrystals embedded in a matrix. X‐ray diffraction measurements have established that the grain size of the matrix material is reduced considerably in these materials. Copyright © 2001 John Wiley & Sons, Ltd.
Characteristics of the uterine luminal surface epithelium at preovulatory and preimplantation stages in the marmoset monkey
Authors: Niklaus Andrea Louise , Murphy Christopher Raymond , Lopata Alexander 
Published: September 2002

AbstractLight and electron microscopy was used to examine the apical luminal epithelial surface of the uterus at preovulatory and preimplantation stages in the marmoset monkey. Luminal surface charge, detected by cationic ferritin staining, progressively decreased from preovulation to day 11 of pregnancy. The smooth, regular apical plasma membrane at preovulatory stages was in contrast to the convoluted, irregular surface observed during early pregnancy, especially at 1 day before blastocyst implantation. Profiles of microvilli were also altered, becoming thicker and more irregular during early pregnancy. Within the epithelial cell body, cyclic morphologic changes were seen, largely in association with secretory organelles. Giant phagocytic bodies were prominent at all stages examined, although their composition and intensity of staining varied throughout the cycle. Weak to moderate estrogen alpha and progesterone receptor immunostaining of the luminal epithelium was found during preovulatory and early pregnancy stages. This study describes complex cyclic changes in the morphology and biochemical make‐up of the uterine luminal epithelial surface in a New World monkey in preparation for blastocyst attachment. Anat Rec 264:82–92, 2001. © 2001 Wiley‐Liss, Inc.
Basic and clinical neuroscience applications of embryonic stem cells
Authors: Gökhan Şölen , Mehler Mark F. 
Published: September 2002

AbstractThere have been recent dramatic advances in our understanding of the molecular mechanisms governing the elaboration of mature tissue‐specific cellular subpopulations from embryonic stem (ES) cells. These investigations have generated a range of new biological and potential therapeutic reagents to allow us to dissect specific stages of mammalian development that were previously experimentally inaccessible. Ultimately, we will be able to reconstitute seminal signaling pathways to promote regeneration of the nervous system. Totipotent ES cells possess an unlimited proliferative capacity that make them attractive candidates for use in a series of innovative transplantation paradigms. Elucidation of the molecular and physiologic properties of ES cells also has important implications for our understanding of the integrative cellular processes underlying neural induction, patterning of the neural tube, neural lineage restriction and commitment, neuronal differentiation, regional neuronal subtype specification, and the specific pathological consequences of alterations in discrete components of these fundamental neurodevelopmental pathways. In addition, recent experimental observations suggest that neurodegenerative disease pathology may involve alterations in a range of progressive neural inductive and neurodevelopmental events through novel biological mechanisms that result in sublethal impairments in cellular homeostasis within evolving regional neuronal precursor populations containing the mutant proteins, culminating in increased vulnerability of their differentiated neuronal progeny to late‐onset apoptosis. Future discoveries in ES cell research will offer unique conceptual and therapeutic perspectives that representing an alternative to neural stem cell therapeutic strategies for ameliorating the pathologic consequences of a broad range of genetic and acquired insults to the developing, adult, and aging brain. Evolving regenerative strategies for both neurodevelopmental and neurodegenerative diseases will likely involve the targeting of vulnerable regional neural precursor populations during “presymptomatic” clinicopathological stages prior to the occurrence of irrevocable neural cell injury and cell death. Anat Rec (New Anat) 265:142–156, 2001. © 2001 Wiley‐Liss, Inc.
Purification and characterization of protein phosphatase‐1 from two cold–hardy goldenrod gall insects
Authors: Pfister Thomas D. , Storey Kenneth B. 
Published: September 2002

AbstractThe catalytic subunit of protein phosphatase‐1 (PP‐1) was purified to homogeneity from final instar larvae (the overwintering stage) of freeze avoiding (Epiblema scudderiana) and freeze tolerant (Eurosta solidaginis) cold‐hardy insects. Arrhenius plots showed that activity of PP‐1 from both species was strongly suppressed at low temperature. Acidic shifts in pH optima and increased inhibition by okadaic acid were also observed when the enzymes were assayed at 4°C compared with 24°C. The data identify multiple ways by which PP‐1 can be inhibited at low temperature and this inhibition appears to be key to sustaining high glycogen phosphorylase activity in support of polyol synthesis at low temperatures. Arch. Insect Biochem. Physiol. 49:56–64, 2002. © 2002 Wiley‐Liss, Inc.
Juvenile hormone catabolism and oviposition in the codling moth, Cydia pomonella, as functions of age, mating status, and hormone treatment
Authors: Cole Tracey J. , Ramaswamy Sonny B. , Srinivasan Asoka , Dorn Silvia 
Published: September 2002

AbstractIn vitro catabolism of juvenile hormone (JH) in haemolymph of adult female Cydia pomonella was ascribed mainly to juvenile hormone esterase (JHE) activity. No significant differences were noted between virgin and mated females 0–96 h post‐emergence. Changes in JHE activity did not appear dependent upon fluctuations in JH titre; conversely, changes in JHE activity could not explain the changes in JH titres. Maximal JHE activity was recorded at 24 h (331.47 ± 47.25 pmol/h/μl; 355.93 ± 36.68 pmol/h/μl, virgin; mated insects, respectively) and preceded the peak in JH titres at 48 h. Topical application of JH II (10 ng–10 μg) or fenoxycarb (50 ng) enhanced JHE activity up to 640 and 56%, respectively. Treatment upon emergence with 10 μg JH II induced enzymic activity for less than 24 h, and when 10 μg JH II or 50 ng fenoxycarb were applied, circulating JH titres returned to control levels within 24 h. Oviposition was highly sensitive to exogenous JH and declined significantly with dosages >100 pg. To allow a degree of oocyte maturation before JH treatment, the hormone was administered at 6, 12, 24, or 48 h post‐emergence and/or females were mated. Neither measure “protected” the system; oviposition declined immediately after JH application. Arch. Insect Biochem. Physiol. 49:10–21, 2002. © 2002 Wiley‐Liss, Inc.
FMRFamide‐like material in the earwig, Euborellia annulipes, and its functional significance
Authors: Rankin Susan M. , Seymour Shannon M. 
Published: September 2002

AbstractThe neurosecretory system of the earwig, Euborellia annulipes, contained material similar to that of FMRFamide, as shown by immunocytochemistry. Within the brain were two pairs of darkly staining perikarya in the medial protocerebrum, and up to four pairs of immunoreactive cells in the lateral protocerebrum. The corpora allata appeared immunoreactive in 10‐day females, but not in 2‐day‐old adults. Additionally, immunoreactive material was detected in midgut endocrine cells of both 2‐ and 10‐day‐old females. FMRFamide at 1 to 100 nM did not inhibit juvenile hormone production by earwig corpora allata in vitro. This was true of glands of low activity from 2‐day cat food–fed or starved virgin females, 10‐day starved females, and those of relatively high activity from 10‐day‐old, cat food–fed females. In contrast, FMRFamide at 50 and 100 (but not at 1) nM stimulated gut motility in vitro in distended guts from 2‐day fed females. Preparations from starved females and those from 10‐day fed females (in which feeding behavior is on the decline) did not respond to exogenous FMRFamide with enhanced rates of contraction. Lastly, preparations from females starved for 7 days and subsequently fed for 3 days responded to 10 nM FMRFamide with increases in gut motility. Arch. Insect Biochem. Physiol. 47:189–197, 2001. © 2001 Wiley‐Liss, Inc.
Developmental roles of platelet‐derived growth factors
Authors: Betsholtz Christer , Karlsson Linda , Lindahl Per 
Published: September 2002

AbstractPlatelet‐derived growth factor (PDGF) was originally identified in platelets and in serum as a mitogen for fibroblasts, smooth muscle cells (SMC) and glia cells in culture. PDGF has since expanded to a family of dimers of at least four gene products, whose biological actions are mediated through two receptor tyrosine kinases, PDGFRs. The present review summarizes and discusses the biological functions of PDGFs and PDGFRs in developmental processes, mainly as revealed through genetic analysis in mice. Such studies have demonstrated multiple critical roles of PDGFs and PDGFRs in embryonic and postnatal development. PDGFs seem to act upon specific populations of progenitor cells that give rise to several different cell types with distinct functions in a variety of developmental processes. Analogies are seen between the cell functions and the developmental processes controlled by PDGFs. This suggests that ancestral PDGF and PDGFR expression patterns and functions may have been iterated in related sets of morphogenetic processes in the course of evolution. BioEssays 23:494–507, 2001. © 2001 John Wiley & Sons, Inc.
Stereoselective degradation of metalaxyl and metalaxyl‐M in soil and sunflower plants
Authors: Marucchini C. , Zadra C. 
Published: September 2002

AbstractA high proportion of agrochemicals are chiral compounds. Since stereoisomers often show different biological and physiological properties, the biological and metabolic responses to these compounds and their fate in the environment are expected to be different. In this work we investigate a possible stereo and/or enantioselective degradation in soil and plants (sunflower) of the fungicide Metalaxyl (rac‐Metalaxyl) and the new compound Metalaxyl‐M ((−)‐(R)‐Metalaxyl) and propose procedures for extraction, cleanup, chromatographic separation of enantiomers, and determination of the R : S ratio by using an HPLC chiral column. The degradation of the two stereoisomers of Metalaxyl proved to be enantioselective and dependent on the media: the (+)‐(S)‐enantiomer showed a faster degradation in plants, while the (−)‐(R)‐enantiomer showed a faster degradation in soil. In this study there was no evidence that racemization of Metalaxyl‐M took place either in soil or in sunflowers. Chirality 14:32–38, 2002. © 2002 Wiley‐Liss, Inc.
Development of an improved automated gas‐chromatographic chiral analysis system: Application to non‐natural amino acids and natural protein hydrolysates
Authors: Nokihara Kiyoshi , Gerhardt Jürgen 
Published: September 2002

AbstractIn the use of combinatorial chemistry as a novel strategy for drug discovery, the chirality assessment of building blocks used for library construction is particularly important in the evaluation of biological actions of generated libraries. The procedure for chiral analysis of lead compounds in screening may be of a high priority, particularly in the case of the protection of intellectual rights. Previously, an automated amino acid analysis system using enantiomer labeling was developed. The system incorporates a reactor, which allows automated esterification and acylation of amino acids, and is connected to an on‐line gas chromatographic system. A capillary column coated with a chiral phase is employed for the separation of the enantiomers. This particular system is improved with a newly constructed “high‐throughput auto‐derivatizer” in combination with a new GC‐system. The resulting data can be processed by newly constructed software. The analyses of amino acid derivatives or hydrolysates of proteins and peptides are carried out routinely within ca. 45 min, including derivatization. Using this system several non‐natural amino acids were tested with respect to the stereoisomeric configuration. In addition, acid hydrolysates of food proteins and tissues obtained by autopsy were analyzed as an application to proteome research. Chirality 13:431–434, 2001. © 2001 Wiley‐Liss, Inc.
Some applications of a chiral fluorometric reagent, (S)‐TBMB carboxylic acid
Authors: Meguro Hiroshi , Kim Jeong‐Hwan , Bai Chen , Nishida Yoshihiro , Ohrui Hiroshi 
Published: September 2002

AbstractMolecular design and applications of a fluorometric chiral agent, (S)‐TBMB carboxylic acid, are briefly reviewed. The agent, possessing an asymmetric 1,3‐benzodioxole skeleton, was designed as a novel class of chiral agent that functions also as a benzoate chromophore for exciton chirality CD methods. The utility of this agent has been demonstrated in an application to determine enantiomeric amino acids, acyl‐sn‐glycerols, glycosyl‐sn‐glycerols, and other chiral alcohols and amines. Chirality 13:441–445, 2001. © 2001 Wiley‐Liss, Inc.
The deep mesencephalic nucleus as an output center of basal ganglia: Morphological and electrophysiological similarities with the substantia nigra
Authors: Rodríguez Manuel , Abdala Patricio , Barroso‐Chinea Pedro , González‐Hernández Tomas 
Published: September 2002

AbstractThe deep mesencephalic nucleus (DMN) is a large midbrain reticular region between the superior colliculus, the substantia nigra compacta, the periaqueductal gray, and the medial geniculate body. Although some data suggest that it is involved in nociception and visceral control, its functions remain unclear. In the present study, by using morphological (combination of anterograde and retrograde tracers with immunocytochemistry and in situ hibrydization) and electrophysiological (firing activity and transynaptic response to striatal stimulation) methods, we show that a subpopulation of DMN cells shares many morphological and electrophysiological characteristics with those of the substantia nigra reticulata (SNR). These similarities include the following: 1) firing rate, firing pattern, and conduction velocity; 2) expression of GAD65, GAD67, and PV; 3) excitatory and inhibitory inputs from the striatum; and 4) projections to the ventral thalamus, superior colliculus, and pedunculopontine tegmental nucleus. Some differences were also found. In comparison with SN, DMN cells and striatal afferents are more sparsely distributed and they show conspicuous contralateral projections to the thalamus and superior colliculus. This suggests that, similarly to the SNR, the DMN acts as an output center of basal ganglia and probably facilitates the inter‐hemispheric regulation of these centers. J. Comp. Neurol. 438:12–31, 2001. © 2001 Wiley‐Liss, Inc.
A mixed finite element for plate bending with eight enhanced strain modes
Authors: Piltner Reinhard , Joseph Deepu S. 
Published: September 2002

AbstractA low‐order thick and thin plate bending element is derived using bilinear approximations for the transverse deflection, the two rotations and the thickness change. The stress–strain relationships from three‐dimensional elasticity are used without any modifications. In order to avoid locking and to improve the accuracy of the results eight enhanced strain modes are used. For an efficient implementation of the mixed element, orthogonal stress and strain functions are utilized. Although the element is a low‐order finite element the numerical results for a series of standard test problems are excellent. Copyright © 2001 John Wiley & Sons, Ltd.