bluuebunny/crossref_metadata_2025_split huggingface dataset:
Somatic and gonadal mosaicism in Hutchinson–Gilford progeria
Authors: Wuyts Wim , Biervliet Martine , Reyniers Edwin , D'Apice Maria Rosaria , Novelli Giuseppe , Storm Katrien 
Published: March 2005

AbstractWe have studied a patient with Hutchinson–Gilford progeria (HGP). Sequence analysis of the LMNA gene demonstrated the presence of a c.1824 C > T (p.G608G) mutation, activating a cryptic splice donor site and leading to the formation of a truncated Lamin A protein. All molecularly characterized autosomal dominant HGP cases described so far result from de novo LMNA mutations, mostly originating on the paternal allele and are often linked with advanced paternal age. However, in our patient, the mutation was transmitted by the mother who showed somatic and germline mosaicism without HGP manifestations. © 2005 Wiley‐Liss, Inc.
Acrofacial dysostosis type Rodríguez
Authors: Dimitrov Boyan , Balikova Irina , Jekova Nely , Vakrilova Lilija , Fryns Jean‐Pierre , Simeonov Emil 
Published: March 2005

AbstractThe acrofacial dysostoses (AFD) are a clinically and causally heterogeneous group of conditions characterized by mandibulofacial dysostosis and a variety of limb anomalies. Several abnormalities affecting different internal organs and the central nervous system (CNS) have been described. Depending on the type of limb defects, two major groups have been delineated: (1) with predominantly pre‐axial anomalies, Nager type AFD, and (2) with predominantly post‐axial involvement, Genée–Wiedemann form of AFD, also known as POADS, respectively. Other forms of “true AFD” have been described as Kelly, Reynolds, Arens (also Tel Aviv form), Rodríguez (or Madrid form), Richieri–Costa, and Patterson–Stevenson–Fontaine types. However, whether they are distinct entities or represent variants of the same condition remains unclear. Rodírguez AFD was described as a new lethal form of AFD in three affected sibs with severe mandibular hypoplasia, severe predominantly pre‐axial limb deficiencies, absent fibulae and ribs, and internal organ anomalies, the most remarkable of which are arrhinencephaly and abnormal lung lobulation. We present a newborn girl with Rodríguez type of AFD, who died a few days after the birth due to respiratory failure. The phenotype and the cause of this condition are discussed. © 2005 Wiley‐Liss, Inc.
Ultrastructural and immunocytochemical studies of the viscacha (Lagostomus maximus maximus) pituitary pars tuberalis
Authors: Perez Romera Edith , Mohamed Fabian , Filippa Verónica , Fogal Teresa , Dominguez Susana , Scardapane Luis , Piezzi Ramón 
Published: March 2005

AbstractThe hypophyseal pars tuberalis (PT) has been the focus of numerous studies attempting to understand its physiological role in the reproductive regulation and modulation by the neuroendocrine system. Ultrastructural studies of the PT in a number of species have shown that it consists of a well‐developed hypophyseal area with important secretory activity, demonstrated by the abundance of secretory granules in the cytoplasm and the marked blood irrigation. This article describes ultrastructural and immunocytochemical aspects of the PT in viscachas captured in their habitat. The cell types identified were PT‐specific cells, agranulated cells, and Folliculostellate cells. PT‐specific cells are divided into type I and II. Type I cells have cytoplasms with secretory granules of 150–500 nm diameter. The secretory granules of type II PT‐specific cells are 65–200 nm in diameter. Both cellular types exhibit numerous nerve endings on the plasmatic membranes. Agranulated cells exhibit nuclei with lax chromatin, mitochondria, phagosomes, scarce Golgi complex, and rough endoplasmic reticulum. Folliculostellate cells exhibit an irregularly shaped and moderately condensed nucleus. All the described cellular types exhibit deposits of cytoplasmic glycogen. The immunocytochemical study revealed the presence of cells immunostained for LH‐β and FSH‐β in the PT caudal zone. ACTH was only detected in the zona tuberalis. No staining was observed with antiprolactin, anti‐TSH‐β, and anti‐GH sera. Folliculostellate cells exhibited staining with anti‐S‐100. The results demonstrate that the viscacha PT is a hypophyseal zone with specific cellular types, which exhibits evident secretory activity. The presence of nerve endings suggests neural control of the function of PT cells. © 2005 Wiley‐Liss, Inc.
Senescence‐associated HSP60 expression in normal human skin fibroblasts
Authors: Di Felice Valentina , Ardizzone Nella , Marcianò Vito , Bartolotta Tommaso , Cappello Francesco , Farina Felicia , Zummo Giovanni 
Published: March 2005

AbstractNormal mammalian fibroblasts cultured in vitro undergo a limited number of divisions before entering a senescent phase in which they can be maintained for long periods but cannot be induced to divide. Senescent cells become unresponsive to growth‐promoting signals and exhibit senescent cell morphology with flattened and enlarged cell shape. Several chaperones have a direct effect on cellular senescence. HSP60 has been largely studied in our laboratories and it has been associated with uncontrolled cell proliferation in tumor cells. Since senescence is firmly regulated during cell cycle progression, we wanted to investigate HSP60 protein level during cellular senescence. Our data show that HSP60 increases during the initial stage of senescence and that it is localized in cellular compartments, resembling mitochondria. An increase in HSP60 protein amount is associated with a cell cycle slow‐down and it may have a role in cell cycle progression. © 2005 Wiley‐Liss, Inc.
Structure and biomechanical properties of the trachea of the striped dolphin Stenella coeruleoalba: Evidence for evolutionary adaptations to diving
Authors: Cozzi Bruno , Bagnoli Paola , Acocella Fabio , Costantino Maria Laura 
Published: March 2005

AbstractThis study analyzes the structure and mechanical properties of the trachea of the striped dolphin Stenella coeruleoalba, one of the most common cetacean species. The cetacean trachea is made up of closed or semiclosed cartilaginous rings without a paries membranaceus. Our results indicate that the inner lining of the trachea contains erectile tissue in which several venous lacunae permeate the mucosa. We also observed and described the presence of peripheral neurons containing nitric oxide along the rim of the venous lacunae. Data obtained from compression and tensile tests and comparison with the pig and goat tracheas indicate a higher stiffness and a different, higher breaking point for the dolphin trachea. On the whole, our data suggest that the trachea of the striped dolphin possesses structural properties that allow rapid filling with blood, possibly in relation to dive activities, and also allow modifications due to increased pressure and immediate return to the original shape without risks of permanent bending or rupture, as would happen in a terrestrial mammal. As the organ undergoes intense pressure difference during descent to optimal foraging depth and subsequent rapid ascent to surface, especially in deep dives of hundreds of meters, the specific structural and biomechanical peculiarities of the trachea of the striped dolphin may represent an evolutionary adaptation to life in the water and to diving. © 2005 Wiley‐Liss, Inc.
New P‐ligands: The aromaticity and reactivity of 2,4,6‐trialkylphenylphospholes
Authors: Keglevich György , Farkas Renáta , Ludányi Krisztina , Kudar Veronika , Hanusz Miklós , Simon Kálmán 
Published: March 2005

AbstractA further member of 2,4,6‐trialkyl‐phenyl‐phos‐pholes, the ditertbutylmethylphenyl derivative (1c) was characterized by Bird index and by the sum of angles at the phosphorus atom to describe the flattening of the P‐pyramid. Both numbers suggested a slight aromaticity. The reaction of arylphospholes with phosphorus tribromide was extended to phosphole 1c leading this occasion, after further steps, to the mixture of 3‐ and 2‐substituted products (3c‐1 and 3c‐2, respectively). A triisopropylphenyl‐2H‐phosphole (4) formed by sigmatropic rearrangement was utilized in the preparation of new 1‐phosphanorbornene derivatives, such as sulfide 6 and hemi‐oxides 8‐1 and 8‐2. Further oxidation of the latter species (8‐1 and 8‐2) led to the decomposition of the dimeric structure (11). 4 could also be trapped by benzaldehyde to afford the oxaphosphanorbornene (10) as one diastereomer. Finally, the reversible formation of 2H‐phosphole 4 from 1H‐phosphole 1a at 150°C was proved. © 2005 Wiley Periodicals, Inc. Heteroatom Chem 16:104–222, 2005; Published online in Wiley InterScience (www.interscience.wiley.com). DOI 10.1002/hc.20077
Regioselective synthesis, structure and behavior in solutions of novel phosphorylated thiazolidin‐4‐ones
Authors: Kozlov Vladimir A. , Odinets Irina L. , Lyssenko Konstantin A. , Churusova Svetlana G. , Yarovenko Sergey V. , Petrovskii Pavel V. , Mastryukova Tatyana A. 
Published: March 2005

AbstractRegioselective syntheses of novel 2‐(phosphoryl)methylidenethiazolidine‐4‐ones 3a–c, 5 by the condensation of phosphoryl acetic acid thioamides 2a–c or substituted thioanilide 4 with dimethyl acetylenedicarboxylate are described. N3‐unsubstituted thiazolidine‐4‐ones 3a–c were obtained as E,Z‐isomers, while N3‐phenyl substituted heterocycle 5 was formed as Z,Z‐isomer. The structures of thiazolidin‐4‐ones 3a‐E,Z and 5‐Z,Z are characterized by crystal structure determination. According to B3Pw91/6‐31G* calculations, the isomers observed in crystals are thermodynamically preferable. In solutions, phosphorylated thiazolidines undergo isomerization (relative to C2 carbon atom of the heterocycle) proceeded by either imine–enamine (N3‐unsubstituted compounds 3a–c) or push–pull mechanisms (N3‐substituted compound 5). © 2005 Wiley Periodicals, Inc. Heteroatom Chem 16:159–222, 2005; Published online in Wiley InterScience (www.interscience.wiley.com). DOI 10.1002/hc.20084
Synthesis, charge distribution, and dimerization behavior of lithium alkynylselenolates
Authors: Pietschnig Rudolf , Merz Klaus , Schäfer Sven 
Published: March 2005

AbstractThe reactivity of alkynyl lithium compounds toward elemental gray selenium yields alkynylselenolates, which show significant interaction with the lithium counter ions, depending on solvent polarity and the electronic nature of the adjacent organic substituent. Likewise the dimerization behavior is different for alkyl‐ and aryl‐substituted alkynylselenolates. From the latter, unsymmetrical 1,3‐selenol is formed, the molecular structure of which was confirmed by X‐ray structure analysis. © 2005 Wiley Periodicals, Inc. Heteroatom Chem 16:169–222, 2005; Published online in Wiley InterScience (www.interscience.wiley.com). DOI 10.1002/hc.20088
Synthesis, spectroscopic characterization, and in vitro biological activity of organotin(IV) complexes of (E)‐3‐(4‐methoxyphenyl)‐2‐phenyl‐2‐propenoic acid
Authors: Sadiq‐ur‐Rehman  , Shahid Khadija , Ali Saqib , Mazhar Mohammad , Badshah Amin , Eng George , Song Xueqing , Ryczkowski Janusz 
Published: March 2005

AbstractOrganotin(IV) complexes with general formula R4 − nSnLn have been synthesized by the reaction of tri‐ and diorganotin chlorides with sodium salt of (E)‐3‐(4‐methoxyphenyl)‐2‐phenyl‐2‐propenoic acid in dry toluene, where RCH3, C2H5, n‐C4H9, C6H5, n = 1 or 2, and L = [ŌOCC(C6H5) CH(C6H4OCH3)]. Dimeric distannoxanes, [(R2SnOOCC(C6H5)CH‐(C6H4OCH3)2O]2, were synthesized by the reaction of acid with diorganotin oxides in 1:1 molar ratio. Multinuclear NMR (1H, 13C, 119Sn), infrared, mass, and Mössbauer spectral techniques have been used to ascertain their structures in solution and as solid. The spectroscopic results showed that triorganotin(IV) derivatives are 4‐coordinated monomers in noncoordinating solvents and 5‐coordinated polymers with bridging carboxylate groups in the solid state. However, diorganotin and dimeric compounds are 5‐coordinated in noncoordinating solvents while in the solid phase may have higher coordination. Biological screening tests showed that most of the compounds have significant activity against different bacteria and fungi. © 2005 Wiley Periodicals, Inc. Heteroatom Chem 16:175–222, 2005; Published online in Wiley InterScience (www.interscience.wiley.com). DOI 10.1002/hc.20089
Spinal and cranial hypertrophic neuropathy in multiple sclerosis
Authors: Quan Dianna , Pelak Victoria , Tanabe Jody , Durairaj Vikram , Kleinschmidt‐Demasters B.K. 
Published: March 2005

AbstractTwo patients with multiple sclerosis developed symptomatic chronic inflammatory demyelinating polyneuropathy with massive spinal or cranial nerve hypertrophy revealed by neuroimaging. Sural nerve biopsy in one showed only moderate demyelination, axonal loss, and onion‐bulb formation, illustrating dichotomy between severe proximal and milder distal nerve involvement. Patients with coexistent central and peripheral demyelination usually are symptomatic from dysfunction at one site or the other, but not from both. Our patients showed minimal response to steroids, intravenous immunoglobulin, or azathioprine. These cases suggest that the mechanism of disease in symptomatic central and peripheral demyelination may differ from that of disease in only one region, and that optimal therapy in this situation must be explored further. Muscle Nerve, 2005
Segmental orientations and deformation mechanism of a poly(ether‐block‐amide) film
Authors: Song Yihu , Nemoto Norio 
Published: March 2005

AbstractSimultaneous measurements of microscopic infrared dichroism, mesoscale deformation, and macroscopic stress have been made for a microphase‐separated film of poly(ether‐block‐amide) 4033 during uniaxial stretching at temperatures between 30 and 91 °C, well below the melting point of the hard polyamide‐12 (PA) domains. Before the onset of dramatic microstructural alterations, the true stress–strain relationship on the mesoscale can be described with an interpenetrating network model, and poly(tetramethylene oxide) (PTMO) soft segments undergo affine deformation. Beyond a threshold strain at which stress from the soft network becomes larger than that from the hard network, plastic deformation occurs in the hard PA domains, and this is accompanied by the downward derivations of the true stress and molecular orientation of PTMO blocks from the model predictions. © 2005 Wiley Periodicals, Inc. J Polym Sci Part B: Polym Phys 43: 1161–1167, 2005
Simulation of workflow balancing in assembly shopfloor operations
Authors: Rajakumar S. , Arunachalam V.P. , Selladurai V. 
Published: March 2005

PurposeTo provide a new model to solve the assembly‐planning problem of a textile machine in a shopfloor which can help researchers and practitioners.Design/methodology/approachThe assembly planning of a textile machine (repetitive manufacturing system) involves the allocation of operations to cross‐trained operators. Workflow is defined as the workloads assigned to the operators. Operators with smaller workloads are selected to be assigned new operations from the list of unscheduled operations. Three different scheduling strategies – random, shortest processing time, and longest processing time – are adopted for the selection of operations to be assigned to operators. Different combinations of these strategies are considered for the selection of both preceding and succeeding operations. A computer simulation program has been coded on an IBM/PC‐compatible system in the C++ language to study the performance of real data from the shopfloor.FindingsThe relative percentage of imbalance is adopted for evaluating the performance of these heuristics. The RL, SL and LL produced well balanced workload schedules with lesser RPI values for all operators other than heuristics.Research limitations/implicationsNon‐traditional approaches like genetic algorithms can be applied to determine the robustness of the results obtained by this research.Practical implicationsThe experiments on simulated and real data clearly indicate that the order of succeeding operations determines the balanced workflows to the assembly of operations among the operators.Originality/valueThe allocation of assembly operations to the operators is modeled into a parallel machine‐scheduling problem with precedence constraints using the objective of minimizing the workflow among the operators.
Topology Optimization of Multicomponent Beam Structure via Decomposition-Based Assembly Synthesis
Authors: Lyu Naesung , Saitou Kazuhiro 
Published: March 2005

AbstractThis paper presents a method for synthesizing multicomponent beam structural assemblies with maximum structural performance and manufacturability. The problem is posed as a relaxation of decomposition-based assembly synthesis, where both topology and decomposition of a structure are regarded as variables over a ground structure with nonoverlapping beams. A multiobjective genetic algorithm with graph-based crossover, coupled with FEM analyses, is used to obtain Pareto optimal solutions to this problem, exhibiting trade-offs among structural stiffness, total weight, component manufacturability (size and simplicity), and the number of joints. Case studies with a cantilever and a simplified automotive floor frame are presented, and representative designs in the Pareto front are examined for the trade-offs among the multiple criteria.
Evidence for Multiple-Antibiotic Resistance in
Campylobacter jejuni
Not Mediated by CmeB or CmeF
Authors: Pumbwe Lilian , Randall Luke P. , Woodward Martin J. , Piddock Laura J. V. 
Published: March 2005

ABSTRACT

An efflux system, CmeABC, in
Campylobacter jejuni
was previously described, and a second efflux system, CmeDEF, has now been identified. The substrates of CmeDEF include ampicillin, ethidium bromide, acridine, sodium dodecyl sulfate (SDS), deoxycholate, triclosan, and cetrimide, but not ciprofloxacin or erythromycin.
C. jejuni
NCTC11168 and two efflux pump knockout strains,
cmeB
::Kan
r
and
cmeF
::Kan
r
, were exposed to 0.5 to 1 μg of ciprofloxacin/ml in agar plates. All mutants arising from NCTC11168 were resistant to ciprofloxacin but not to other agents and contained a mutation resulting in the replacement of threonine 86 with isoleucine in the quinolone resistance-determining region of GyrA. Mutants with two distinct phenotypes were selected from the efflux pump knockout strains. Mutants with the first phenotype were resistant to ciprofloxacin only and had the same substitution within GyrA as the NCTC11168-derived mutants. Irrespective of the parent strain, mutants with the second phenotype were resistant to ciprofloxacin, chloramphenicol, tetracycline, ethidium bromide, acridine orange, and SDS and had no mutation in
gyrA
. These mutants expressed levels of the efflux pump genes
cmeB
and
cmeF
and the major outer membrane protein gene
porA
similar to those expressed by the respective parent strains. No mutations were detected in
cmeF
or
cmeB
. Accumulation assays revealed that the mutants accumulated lower concentrations of drug. These data suggest the involvement of a non-CmeB or -CmeF efflux pump or reduced uptake conferring multiple-antibiotic resistance, which can be selected after exposure to a fluoroquinolone.
Pseudomonas aeruginosaBloodstream Infection: Importance of Appropriate Initial Antimicrobial Treatment
Authors: Micek Scott T. , Lloyd Ann E. , Ritchie David J. , Reichley Richard M. , Fraser Victoria J. , Kollef Marin H. 
Published: March 2005

ABSTRACTPseudomonas aeruginosabloodstream infection is a serious infection with significant patient mortality and health-care costs. Nevertheless, the relationship between initial appropriate antimicrobial treatment and clinical outcomes is not well established. This study was a retrospective cohort analysis employing automated patient medical records and the pharmacy database at Barnes-Jewish Hospital. Three hundred five patients withP. aeruginosabloodstream infection were identified over a 6-year period (January 1997 through December 2002). Sixty-four (21.0%) patients died during hospitalization. Hospital mortality was statistically greater for patients receiving inappropriate initial antimicrobial treatment (n= 75) compared to appropriate initial treatment (n= 230) (30.7% versus 17.8%;P= 0.018). Multiple logistic regression analysis identified inappropriate initial antimicrobial treatment (adjusted odds ratio [AOR], 2.04; 95% confidence interval [CI], 1.42 to 2.92;P= 0.048), respiratory failure (AOR, 5.18; 95% CI, 3.30 to 8.13;P< 0.001), and circulatory shock (AOR, 4.00; 95% CI, 2.71 to 5.91;P< 0.001) as independent determinants of hospital mortality. Appropriate initial antimicrobial treatment was administered statistically more often among patients receiving empirical combination antimicrobial treatment for gram-negative bacteria compared to empirical monotherapy (79.4% versus 65.5%;P= 0.011). Inappropriate initial empirical antimicrobial treatment is associated with greater hospital mortality among patients withP. aeruginosabloodstream infection. Inappropriate antimicrobial treatment ofP. aeruginosabloodstream infections may be minimized by increased use of combination antimicrobial treatment until susceptibility results become known.
Activity of Hoechst 33258 against
Pneumocystis carinii
f. sp.
muris
,
Candida albicans
, and
Candida dubliniensis
Authors: Disney Matthew D. , Stephenson Ruth , Wright Terry W. , Haidaris Constantine G. , Turner Douglas H. , Gigliotti Francis 
Published: March 2005

ABSTRACT

Hoechst 33258 is a compound that binds nucleic acids. We report that Hoechst 33258 exhibits antimicrobial activity against
Pneumocystis carinii
f. sp.
muris
in a mouse model for
P. carinii
pneumonia and against
Candida albicans
and
Candida dubliniensis
in vitro. Relative to saline treatment, a 14-day, daily treatment of mice with 37.5 mg of Hoechst 33258/kg of body weight after inoculation with
P. carinii
reduced by about 100-fold the number of
P. carinii
organisms detected by either PCR or by microscopy after silver staining. For comparison, treatment based on a dose of 15 to 20 mg of the trimethoprim component in trimethoprim-sulfamethoxazole/kg reduced the number of
P. carinii
by about fourfold. In vitro inhibition of
P. carinii
group I intron splicing was observed with a 50% inhibitory concentration (IC
50
)of 30 μM in 2 or 4 mM Mg
2+
, suggesting RNA as a possible target. However, Hoechst 33258 inhibits growth of
Candida
strains with and without group I introns. IC
50
s ranged from 1 to 9 μM for strains with group I introns and were 12 and 32 μM for two strains without group I introns. These studies demonstrate that compounds that bind fungal nucleic acids have the potential to be developed as new therapeutics for
Pneumocystis
and possibly other fungi, especially if they could be directed to structures that are not present in mammalian cells, such as self-splicing introns.