bluuebunny/crossref_metadata_2025_split huggingface dataset:
New sporadic case of congenital dyserythropoietic anemia type III in an aged woman: detailed description of ultrastructural findings
Authors: Sigler Erica , Shaft Dina , Shtalrid Mordechai , Shvidel Lev , Berrebi Alain , Resnitzky Peretz 
Published: August 2002

AbstractWe describe a new case of congenital dyserythropoietic anemia (CDA) type III. This least common type of CDA was diagnosed at the age of 59 in a 70‐year‐old woman who suffered from a young age from mild macrocytic anemia, while the long follow up since diagnosis documented a benign clinical course. No family history of blood diseases was obtained and no anemia was documented in the medical records of any of her four children. The bone marrow (BM) examination on light microscopy revealed a severe erythroid hyperplasia with the presence of giant multinucleated erythroblasts. Ultrastructural examination of the BM disclosed the presence of many large multinucleated erythroblasts bearing a variety of ultrastructural findings: nuclear clefts, autophagic vacuoles, iron‐loaded mitochondria, and intracytoplasmic myelin figures. In addition, extensive hyperlobulation of the nucleus and partial loss of nuclear membrane with “spilling” of nuclear material to the adjacent cytoplasm was also noted in some of the erythroblasts. These last two findings have not been previously described in CDA III. Am. J. Hematol. 70:72–76, 2002. © 2002 Wiley‐Liss, Inc.
Genetic heterogeneity of primary familial and congenital polycythemia
Authors: Kralovics Robert , Prchal Josef T. 
Published: August 2002

AbstractPrimary familial and congenital polycythemia (PFCP) is an inherited disorder of erythroid progenitor cells resulting in elevated erythrocyte mass. Several mutations of the erythropoietin receptor (EPOR) gene have been associated with PFCP, although in a few families the linkage between the EPOR gene and PFCP has been excluded. To examine the role of EPOR mutations in the pathogenesis of PFCP, we studied 43 unrelated PFCP subjects. Erythroid culture data were available in 26 subjects, and in all these subjects, we observed hypersensitivity of erythroid progenitors to erythropoietin (EPO). We screened all EPOR gene exons for mutations using ribonuclease cleavage assay and protein truncation test. We detected five mutations in exon VIII of the EPOR gene, four of which we reported earlier. A new EPOR gene mutation was found (G5959T) that changes codon 425 GAG to a termination codon, resulting in truncation of the EPOR by 84 amino acids. The G5959T mutation was found to segregate with the disease in the affected family and represents another example of a nonsense mutation associated with PFCP. We also report the first intronic mutation (A2706T) of the EPOR gene. The finding of only five disease‐causing mutations in our PFCP patient pool of 43 subjects (12%) indicates that EPOR gene mutations are not the major genetic defect associated with PFCP. The hypersensitivity of erythroid progenitors to EPO seen in all examined PFCP subjects suggests a dominant lesion of an as yet unidentified gene either at the level of the EPOR signaling pathway or another erythropoiesis regulating pathway. Am. J. Hematol. 68:115–121, 2001. © 2001 Wiley‐Liss, Inc.
Age change of power in weight/heightp indices used as indicators of adiposity in japanese
Authors: Hattori Komei , Hirohara Toshie 
Published: August 2002

AbstractData from the Statistical Report on School Health and Hygiene were used to identify the entire process of the power of height p in the weight‐for‐height index W/Hp. The appropriate power of height p was determined by clarifying the allometric relationship, giving an index of relative weight, which is highly correlated with weight but uncorrelated with height for age. The procedure is equivalent to fitting a straight line on a log–log scale to the tabulated weights by height in different age groups. The p values show directional changes from 5 to 17 years of age in both sexes. The optimal p value is approximately 2 in preschool children but increases gradually to over 3 at 10 years in males and 11 years in females, and then falls back to 2 after puberty. The largest p value occurs 18 months earlier in females than in males, and the pattern is the same in the present samples and American samples. Because there are remarkable changes between 12 to 14 years in females and 14 to 16 years in males, the general weight‐for‐height indices with the precise power of height are preferable at these ages. A fixed power 3.0 is suggested for children between 3 and 12 years irrespective of sex or race. Am. J. Hum. Biol. 14:275–279, 2002. © 2002 Wiley‐Liss, Inc.
Deletion of RBM and DAZ in azoospermia: Evaluation by PRINS
Authors: Kadandale Jayarama S. , Wachtel Stephen S. , Tunca Yusuf , Martens Paula R. , Wilroy R. Sid , Tharapel Avirachan T. 
Published: August 2002

AbstractMolecular and cytogenetic studies from infertile men have shown that one or more genes controlling spermatogenesis are located in proximal Yq11.2 in interval 6 of the Y chromosome. Microdeletions within the azoospermia factor region (AZF) are often associated with azoospermia and severe oligospermia in men with idiopathic infertility. We evaluated cells from a normal‐appearing 27‐year‐old man with infertility and initial karyotype of 45,der(X)t(X;Y)(p22.3;p11.2)[8]/46,t(X;Y)(p22.3;p11.2)[12]. By fluorescence in situ hybridization with dual‐color whole chromosome paint probes for X and Y chromosomes, we confirmed the Xp‐Yp interchange. By primed in situ labeling, we identified translocation of the SRY gene from its original location on Yp to the patient's X chromosome at band Xp22. We also obtained evidence that the apparent marker was a der(Y) (possibly a ring) containing X and Y domains, and observed that the patient's genome was deleted for RBM and DAZ, two candidate genes for AZF. © 2001 Wiley‐Liss, Inc.
Problem behavior in boys with fragile X syndrome
Authors: Hatton Deborah D. , Hooper Stephen R. , Bailey Donald B. , Skinner Martie L. , Sullivan Kelly M. , Wheeler Anne 
Published: August 2002

AbstractThis study examines problem behavior over time in 59 boys with fragile X syndrome (FXS), aged 4–12 years, using the Child Behavior Checklist (CBCL). Approximately 49% of the boys scored within the borderline or clinical range on total problem behavior, while 56–57% scored in the borderline or clinical range on the attention and thought problems subscales, and 26% scored in this range on the social problems subscale. With a mean of 2.5 assessments per child, behavior problems were stable during the 3‐year period of study. Total problem behavior was higher for children who displayed autistic behavior, were rated as low in adaptability, had mothers with higher maternal education levels, and were on medication. Mothers with more education also rated their children as having more attention, thought, and total problems. Children taking medication differed from boys who were not taking medication on social problems, but not on attention and thought problems. Low adaptability and more autistic characteristics predicted thought problems. © 2002 Wiley‐Liss, Inc.
Benefits of polyspecific associations for the Goeldi’s monkey (Callimico goeldii)
Authors: Porter Leila M. 
Published: August 2002

AbstractPolyspecific associations are an important component of Callimico goeldii behavior and ecology. On average, Callimico goeldii was found in proximity to or in vocal contact with Saguinus troops (S. fuscicollis and S. labiatus) during 53% of all time intervals sampled. Polyspecific associations varied considerably between seasons, however, with association rates peaking during the wet‐season month of February (89%) and declining in the dry season, with the lowest rate (13%) in July. The primary benefits of associations appear to be an increased use of the lower and middle canopy, and an increase in feeding behaviors during the wet season. Thus, Callimico goeldii appear to benefit most from associations during the wet season when fruits are its principal food source. Fruits are eaten more in the forest canopy than in the understory; thus, an increase in height use likely permits an increase in the fruit resources on which Callimico goeldii can forage and feed. In addition, Saguinus groups, with their smaller home ranges, are likely to be more knowledgeable than Callimico goeldii about the location and abundance of ripe fruits in their home ranges. Thus, Callimico goeldii may parasitize Saguinus for their fruit knowledge by following them through their ranges. In the dry season, limited dietary overlap between Callimico goeldii and Saguinus groups is likely to make associations less beneficial for Callimico goeldii as they adopt different foraging and ranging strategies. Am. J. Primatol. 54:143–158, 2001. © 2001 Wiley‐Liss, Inc.
Rib lesions in a prehistoric Puebloan sample from southwestern Colorado
Authors: Lambert Patricia M. 
Published: August 2002

AbstractAn unusually high frequency of periosteal lesions of visceral rib surfaces was observed in a small, prehistoric skeletal series from southwestern Colorado. Lesions of this type have been concordant with pulmonary tuberculosis in three studies of human skeletal collections with known cause of death, and in a recent clinical investigation of rib dimensions in living patients with lung disorders. Diseases such as pneumonia and actinomycosis have also been found to cause these lesions, but in much lower frequencies. Archaeological evidence suggests that Puebloan farmers of Sleeping Ute Mountain's southern piedmont, from which the sample is drawn, endured unusually harsh environmental conditions punctuated by severe drought and exacerbated by escalating warfare. It is argued here that these environmental stressors increased susceptibility to an opportunistic respiratory infection reminiscent of tuberculosis, and possibly also some form of pneumonia, resulting in high rates of active disease previously noted only in historic Puebloan peoples. Am J Phys Anthropol 117:281–292, 2002. © 2002 Wiley‐Liss, Inc.
Sodium channel inactivation in an animal model of acute quadriplegic myopathy
Authors: Rich Mark M. , Pinter Martin J. 
Published: August 2002

AbstractWe previously demonstrated that muscle fibers become unable to fire action potentials in both patients and an animal model of acute quadriplegic myopathy (AQM). In the animal model, skeletal muscle is denervated in rats treated with high‐dose corticosteroids (steroid‐denervated; SD), and muscle fibers become inexcitable despite resting potentials and membrane resistances similar to those of control denervated fibers that remain excitable. We show here that unexcitability of SD fibers is due to increased inactivation of sodium channels at the resting potential of affected fibers. A hyperpolarizing shift in the voltage dependence of inactivation in combination with the depolarization of the resting potential induced by denervation results in inexcitability. Our findings suggest that paralysis in the animal model of AQM is the result of an abnormality in the voltage dependence of sodium channel inactivation.
Peroxisome proliferator‐activated receptor‐γ agonists prevent experimental autoimmune encephalomyelitis
Authors: Feinstein Douglas L. , Galea Elena , Gavrilyuk Vitaliy , Brosnan Celia F. , Whitacre Caroline C. , Dumitrescu‐Ozimek Lucia , Landreth Gary E. , Pershadsingh Harrihar A. , Weinberg Guy , Heneka Michael T. 
Published: August 2002

AbstractThe development of clinical symptoms in multiple sclerosis and its animal model experimental autoimmune encephalomyelitis (EAE) involves T‐cell activation and migration into the central nervous system, production of glial‐derived inflammatory molecules, and demyelination and axonal damage. Ligands of the peroxisome proliferator‐activated receptor (PPAR) exert anti‐inflammatory effects on glial cells, reduce proliferation and activation of T cells, and induce myelin gene expression. We demonstrate in two models of EAE that orally administered PPARγ ligand pioglitazone reduced the incidence and severity of monophasic, chronic disease in C57BL/6 mice immunized with myelin oligodendrocyte glycoprotein peptide and of relapsing disease in B10.Pl mice immunized with myelin basic protein. Pioglitazone also reduced clinical signs when it was provided after disease onset. Clinical symptoms were reduced by two other PPARγ agonists, suggesting a role for PPARγ activation in protective effects. The suppression of clinical signs was paralleled by decreased lymphocyte infiltration, lessened demyelination, reduced chemokine and cytokine expression, and increased inhibitor of kappa B (IkB) expression in the brain. Pioglitazone also reduced the antigen‐dependent interferon‐γ production from EAE‐derived T cells. These results suggest that orally administered PPARγ agonists could provide therapeutic benefit in demyelinating disease.
A novel deafness/dystonia peptide gene mutation that causes dystonia in female carriers of Mohr‐Tranebjaerg syndrome
Authors: Swerdlow Russell H. , Wooten G. Frederick 
Published: August 2002

AbstractSex‐linked male deafness and dystonia (Mohr‐Tranebjaerg syndrome) arises from mutation of the deafness/dystonia peptide (DDP) gene. We describe a novel guanine deletion at nucleotide 108 of the DDP gene in a family with Mohr‐Tranebjaerg syndrome, which terminates this 97–amino acid protein at codon 25. Unlike previously reported kindreds, carrier females in this family also manifest dystonias, including torticollis and writer's cramp. A family history of male deafness should alert clinicians to the possibility of DDP mutation in women with focal dystonias.
Synthesis and characterization of poly(N‐vinylpyrrolidone) filled by monodispersed silver clusters with controlled size
Authors: Carotenuto G. 
Published: August 2002

AbstractDispersions of very small metal particles in polymeric matrixes are scientifically and technologically important. Here, poly(N‐vinylpyrrolidone) (PVP) filled with nanometric silver particles characterized by a narrow size distribution and a controlled average dimension has been obtained using a modification of the polyol process. In particular, the nanocomposite material was prepared by reduction of silver nitrate in ethylene glycol in the presence of ultrasound and PVP as a protective agent. The final particle size was controlled by removing the silver–PVP system from the reactive mixture by addition of acetone. The UV–visible spectrum of the material shows a very strong plasmon resonance band centred at 410 nm. The band position depends on the particle size and, consequently, the control of the particle growth process allows one to modulate the nanocomposite absorption wavelength. Such an important characteristic offers the possibility to use Ag–PVP nanocomposites to produce UV‐absorbers and colour filters for advanced optical devices. Copyright © 2001 John Wiley & Sons, Ltd.
DNA damage induced by organotins on ­trout‐nucleated erythrocytes
Authors: Tiano Luca , Fedeli Donatella , Moretti Massimo , Falcioni Giancarlo 
Published: August 2002

AbstractThe effect of tributyltin‐chloride (TBTC), dibutyltin‐chloride (DBTC) and monobutyltin‐chloride (MBTC) on rainbow trout (Salmo irideus) nuclear DNA, was investigated by means of single cell gel electrophoresis (‘comet’ assay). Our data show that TBTC presents a marked genotoxic effect, whereas the genotoxic effect is less pronounced for DBTC and it is completely absent for MBTC. These results could be important in evaluating the environmental risks deriving from the use of these molecules as a antifouling agents in marine paints and as agricultural biocides. Copyright © 2001 John Wiley & Sons, Ltd.
Abbreviations:

TBTC,

tributyltin‐chloride;

DBTC,

dibutyltin‐chloride;

MBTC,

monobutyltin‐chloride;

Hb,

hemoglobin.
Effect of different organotin compounds on DNA of gilthead sea bream (Sparus aurata) erythrocytes assessed by the comet assay
Authors: Gabbianelli Rosita , Villarini Milena , Falcioni Giancarlo , Lupidi Giulio 
Published: August 2002

AbstractThe ‘comet’ assay appears to be a promising tool for estimating DNA damage at the single cell level. We used this test to evaluate the effect of organotin compounds on sea bream nucleated erythrocytes. The tributyltin chloride (TBTC), dibutyltin chloride (DBTC) and monobutyltin chloride (MBTC) employed in this study show different genotoxicities. TBTC and DBTC have pronounced effects on tail length, tail intensity and tail moment, though TBTC is more efficient in producing DNA damage. MBTC leads to a fast genotoxic effect that does not change with the incubation time. The data obtained are important for the analysis of the environmental risks produced by organotin compounds used as antifouling agents in marine paints and as biocides in agriculture. Copyright © 2002 John Wiley & Sons, Ltd.
Vinyl‐containing silicone resin as the crosslinking agent of heat‐curable silicone rubber
Authors: Zhao Shigui , Feng Shengyu 
Published: August 2002

AbstractHeat‐curable silicone rubber (HCSR) was prepared by using vinyl‐containing silicone resin (VSR) as the crosslinking agent instead of polyvinylsilicone oil (C gum). Mechanical properties and crosslink density of the vulcanizates were measured. The results indicate that VSR is a good crosslinking agent for HCSR. The tensile strength, tearing strength, elongation at break, and hardness of the vulcanizate can reach 10.2 MPa, 29.1 kN/m, 720%, and 58 SHA, respectively. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 83: 3123–3127, 2002; DOI 10.1002/app.10054
Preparation and characterization of poly(p‐phenylenediamine‐co‐xylidine)
Authors: Li Xin‐Gui , Huang Mei‐Rong , Chen Run‐Feng , Jin Yi , Yang Yu‐Liang 
Published: August 2002

Abstractp‐Phenylenediamine (PPDA) homopolymer and its copolymers with 2,3‐xylidine (XY) were synthesized by oxidative polymerization using potassium persulfate as an oxidant in HCl medium at room temperature. The yield, intrinsic viscosity, and solubility of the polymers were significantly dependent on the monomer ratio. The resulting polymers were characterized by Fourier transform IR spectroscopy, 1H‐NMR spectroscopy, wide‐angle X‐ray diffraction, and thermogravimetry methods. The results showed that the number‐average degree of polymerization of the PPDA homopolymer was 33 and the actual content of XY units in the copolymer was slightly higher than the feed XY unit content. The polymers were substantially amorphous and showed the strongest diffraction at a Bragg angle of 3°. The polymers exhibited a thermal decomposition temperature higher than 436°C, the maximum weight‐loss rate was slower than 4%/min, and the char yield was larger than 24 wt % at 600°C in nitrogen. The activation energy of thermal decomposition for the polymers increased from 19 to 25 kJ/mol with increasing XY unit contents from 0 to 10 mol %. The polymers showed higher thermostability but lower activation energy of decomposition in nitrogen than in air. © 2001 John Wiley & Sons, Inc. J Appl Polym Sci 81: 3107–3116, 2001
Synthesis of polymer particles with specific lysozyme recognition sites by a molecular imprinting technique
Authors: Hirayama Kazuko , Sakai Yoshiro , Kameoka Kei 
Published: August 2002

AbstractTo prepare silica beads covered with a lysozyme‐imprinted polymer layer, we polymerized acrylamide and acrylic acid or acrylamide and N,N‐dimethylaminopropylacrylamide with (NH4)2S2O8 in a phosphate buffer containing the lysozyme, surface‐modified silica beads, and crosslinkers; the result was the formation of a polymer layer with a lysozyme recognition site on the silica‐bead surface. By quantitative analysis of the supernatant of the solution containing the silica beads, we confirmed that modified silica beads, in contrast to unmodified silica beads, can selectively adsorb lysozymes. The process of binding and releasing the lysozyme to and from the modified silica beads can be repeated several times without degradation of the rebinding ability. A quartz‐crystal microbalance sensor fabricated with a molecularly imprinted polymer layer with a lysozyme recognition site was prepared. When a lysozyme aqueous solution was added to the solution in which the sensor was immersed, a high level of sensitivity and response was observed. High selectivity was also demonstrated by tests with other protein solutions. © 2001 John Wiley & Sons, Inc. J Appl Polym Sci 81: 3378–3387, 2001
Secretion by striated ducts of mammalian major salivary glands: Review from an ultrastructural, functional, and evolutionary perspective
Authors: Tandler Bernard , Gresik Edward W. , Nagato Toshikazu , Phillips Carleton J. 
Published: August 2002

AbstractIn addition to their role in electrolyte homeostasis, striated ducts (SDs) in the major salivary glands of many mammalian species engage in secretion of organic products. This phenomenon usually is manifested as the presence of small serous‐like secretory granules in the apical cytoplasm of SD cells. The composition of these granules is largely unknown, except in the case of the cat and rat submandibular gland, where the granules have unequivocally been shown to contain kallikrein. In some species, the apical cytoplasm of SD cells contains variable numbers of vesicles, both spherical and elongated, that vary in appearance from ‘empty’ to moderately dense. In the rat parotid gland, lucent vesicles transport glycoproteins to the luminal surface where they are incorporated into the apical plasmalemma and the glycocalyx. There is a strong possibility that in various species some of these vesicles are involved in transcytosis of antibodies to the saliva from their source (plasma cells) in the surrounding connective tissue. In addition, vesicles may engage in transfer of growth factors from the saliva to the interstitium. In a few species, conventional SDs have been replaced by ducts that are wholly given over to secretion, i.e., they entirely lack basal striations; although such ducts occupy the histological position of conventional SDs, it is not clear whether they represent a new type of duct or merely are modifications of SDs. Broad‐based comparisons of ultrastructural and other data about SDs offer some insight into evolutionary history of salivary glands and their role in the adaptive radiation of mammals. Evolutionary patterns emerged when we made interspecific comparisons across mammalian orders. Among the bats, there is a clear relationship between SD secretion and general categories of diet. Anat Rec 264:121–145, 2001. © 2001 Wiley‐Liss, Inc.
Third instar nymphs of Rhodnius prolixus exposed to α‐cyanopyrethroids: From hyperactivity to death
Authors: Alzogaray Raúl A. , Zerba Eduardo N. 
Published: August 2002

AbstractThe hyperactivity, incoordination, recovery, and mortality produced by four α‐cyanopyrethroids usually used for Chagas disease vector control (β‐cypermethrin, β‐cyfluthrin, λ‐cyhalothrin, and deltamethrin) were evaluated on third instar nymphs of Rhodnius prolixus. All pyrethroids modified the locomotor activity of the nymphs, which increased linearly as a function of the log of insecticide concentration. λ‐Cyhalothrin showed the lowest values of Effective Concentration 50%, Lethal Concentration 50%, Effective Time 50%, and Lethal Time 50% when insecticides were applied by contact with treated filter papers. Recovery from incoordination was observed after topical application of the insecticides. The recovery was inhibited by the simultaneous application of piperonyl butoxide, suggesting that biotransformation by mixed‐function microsomal oxidases is involved in the process of recovery. Arch. Insect Biochem. Physiol. 46:119–126, 2001. © 2001 Wiley‐Liss, Inc.