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51.
52.
Protective protein/cathepsin A (PPCA) is a pleiotropic lysosomal enzyme that complexes with beta-galactosidase and neuraminidase, and possesses serine carboxypeptidase activity. Its deficiency in man results in the neurodegenerative lysosomal storage disorder galactosialidosis (GS). The mouse model of this disease resembles the human early onset phenotype and results in severe nephropathy and ataxia. To understand better the pathophysiology of the disease, we compared the occurrence of lysosomal PPCA mRNA and protein in normal adult mouse tissues with the incidence of lysosomal storage in PPCA(-/-) mice. PPCA expression was markedly variable among different tissues. Most sites that produced both mRNA and protein at high levels in normal mice showed extensive and overt storage in the knockout mice. However, this correlation was not consistent as some cells that normally expressed high levels of PPCA were unaffected in their storage capability in the PPCA(-/-) mice. In addition, some normally low expressing cells accumulated large amounts of undegraded products in the GS mouse. This apparent discrepancy may reflect a requirement for the catalytic rather than the protective function of PPCA and/or the presence of cell-specific substrates in certain cell types. A detailed map showing the cellular distribution of PPCA in nomal mouse tissues as well as the sites of lysosomal storage in deficient mice is critical for accurate assessment of the effects of therapeutic interventions.  相似文献   
53.
STUDY OBJECTIVE: To investigate the effect of Pseudomonas aeruginosa infection on clinical parameters in Chinese patients with noncystic fibrosis and steady-state bronchiectasis. DESIGN: Prospective, cross-sectional clinicomicrobiological study with informed consent. SETTING: Consecutive outpatient recruitment from a specialist bronchiectasis respiratory clinic. PATIENTS: Outpatients (n = 100; 62 women; 55.1+/-16.7 years old; FEV1/FVC 1.4+/-0.7/2.1+/-0.9 L), who had stable respiratory symptoms for more than 3 weeks. MEASUREMENTS AND RESULTS: Respiratory pathogens isolated from the sputum were: Pseudomonas aeruginosa (33), Haemophilus influenzae (10), Moraxella catarrhalis (2), other Gram-negative bacilli (5), Streptococcus pneumoniae (6), Staphylococcus aureus (5), mycobacteria (3), and yeast (1). Clinical parameters in patients with positive isolation of P aeruginosa were compared with those without the organism in the sputum culture (non-P aeruginosa). In the P aeruginosa group, the FEV1/FVC ratio and sputum volume were lower (p < 0.005) and higher (p < 0.0001), respectively, than those of the non-P aeruginosa group. The FEV1/FVC ratio (< 60%) and sputum volume (grading > 5) were independently associated with a positive sputum isolation of P aeruginosa with odds ratios of 3.1 (confidence interval [CI] 1.2 to 8.4; p < 0.01) and 4.7 (CI 1.6 to 13.3; p < 0.001), respectively. CONCLUSIONS: P aeruginosa is the predominant respiratory pathogen isolated in the sputum of Chinese patients with steady-state bronchiectasis, and its isolation is associated with high sputum output (> or = 75th quartile) and moderately severe airflow obstruction (FEV1/FVC < 60%).  相似文献   
54.
Lactation records (n = 2362) of 1402 crossbred cows in 22 cooperating dairy herds in southeastern Brazil were evaluated. Cows were mixtures of Zebu (Gir, Guzera, and unknown) and European breeding (mostly Holstein). Lactation milk yields were expressed as total, 3050-d, or deviated 305-d yields, either adjusted or unadjusted for days in milk (DIM). Mean DIM was 280. Arithmetic means unadjusted for DIM were 1942, 1666, and 5 kg per record. Milk yields of daughters from sires of 6/8 and 7/8 European breeds were higher than yields of daughters from sires of 5/8 European breeds when data were either adjusted or unadjusted for DIM. The differences associated with breed group of sire were only slightly reduced when records were adjusted for DIM. There was no evidence of a decline in milk yield as the fraction of European breeding of the sire increased from 6/8 to 7/8. For a given breed group of sire, whether the grandsire was purebred or crossbred had no detectable effect. These results should be useful in determining strategies for crossbreeding of dairy cows in tropical areas, particularly when crossbred sires are used.  相似文献   
55.
The concept of a fracture process zone where damage takes place is used to analyse the delaminations at the free edges of angle ply laminates under uniaxial tension. The use of a fracture process zone removes the singularity in the interlaminar stresses and enables the initiation and growth of delaminations to be modelled for a perfect laminate without any assumed prior defects. Two different models for the stress displacement relationship in the fracture process zone are examined: a constant stress up to critical displacement and a linear relationship. Finite element analysis shows that there is little difference in the predictions obtained from the two models. An approximate analysis is presented for the constant stress stress-displacement model which is shown to agree with a finite element solution and experimental data. Hence it is argued that the approximate method using a constant stress model for the fracture process zone is sufficient for accurate prediction of delaminations.  相似文献   
56.
This paper is concerned with the problem of positive observer synthesis for positive systems with both interval parameter uncertainties and time delay. Conventional observers may no longer be applicable for such kind of systems due to the positivity constraint on the observers, and they only provide an estimate of the system state in an asymptotic way. A pair of positive observers with state‐bounding feature is proposed to estimate the state of positive systems at all times in this paper. A necessary and sufficient condition for the existence of desired observers is first established, and the observer matrices can be obtained easily through the solutions of a set of linear matrix inequalities (LMIs). Then, to reduce the error signal between the system state and its estimates, an iterative LMI algorithm is developed to compute the optimized state‐bounding observer matrices. Finally, a numerical example is presented to show the effectiveness and applicability of the theoretical results. Copyright © 2011 John Wiley & Sons, Ltd.  相似文献   
57.
Reflective displays are advantageous in applications requiring low power or daylight readability. However, there are no low‐cost reflective technologies capable of displaying bright colors. By employing photoluminescence to more efficiently use ambient light, we created a prototype display that provides bright, full color in a simple, low‐cost architecture. This prototype includes a novel electrokinetic shutter, a layer that incorporates patterned luminescent red, green, and blue sub‐pixel elements, and a novel optical out‐coupling scheme. The luminescent elements convert otherwise‐wasted portions of the incident spectrum to light in the desired color band, resulting in improved color saturation and lightness. This prototype provides a color gamut that is superior to competing reflective display technologies that utilize color filters in single‐layer side‐by‐side sub‐pixel architectures. The current prototype is capable of switching in <0.5 s; future displays based on an alternative electro‐optic shutter technology should achieve video rate operation. A transflective version of this technology has also been prototyped. The transflective version utilizes its backlight with a power efficiency that is at least three times that of a conventional liquid crystal display. These photoluminescence‐based technologies enable a host of applications ranging from low‐power mobile products and retail pricing signage to daylight readable signage for outdoor advertising segments.  相似文献   
58.
Insulator-based dielectrophoresis (iDEP) has been successfully used for on-chip manipulations of biological samples. Despite its effectiveness, iDEP typically requires high DC voltages to achieve sufficient electric field; this is mainly due to the coupled phenomena among linear electrokinetics: electroosmosis (EO) and electrophoresis (EP) and nonlinear electrokinetics: dielectrophoresis (DEP). This paper presents a microfluidic technique using DC-offset AC electric field for electrokinetic concentration of particles and cells by repulsive iDEP. This technique introduces AC electric field for producing iDEP which is decoupled from electroosmosis (EO) and electrophoresis (EP). The repulsive iDEP is generated in a PDMS tapered contraction channel that induces non-uniform electric field. The benefits of introducing AC electric field component are threefold: (i) it contributes to DEP force acting on particles, (ii) it suppresses EO flow and (iii) it does not cause any EP motion. As a result, the required DC field component that is mainly used to transport particles on the basis of EO and EP can be significantly reduced. Experimental results supported by numerical simulations showed that the total DC-offset AC electric field strength required to concentrate 15-μm particles is significantly reduced up to 85.9% as compared to using sole DC electric field. Parametric experimental studies showed that the higher buffer concentration, larger particle size and higher ratio of AC-to-DC electric field are favorable for particle concentration. In addition, the proposed technique was demonstrated for concentration of yeast cells.  相似文献   
59.
The rising production of produced water from oilfields had been proven to bring detrimental environmental effects. In this study, an efficient, recyclable, and environmental-friendly reduced graphene oxide immobilized κ-Carrageenan hydrogel composite(κCa GO) was fabricated as an alternative sorbent for crude oil-in-water demulsification. Polyethyleneimine(PEI) was employed to form a stable hydrogel composite. The conditions for the immobilization of graphene oxide(GO) on PEI-modified κ-Carrageenan(κC) beads were optimized appropriately. An immobilization yield of 77% was attained at 2% PEI, 2 h immobilization activation time, and p H6.5. Moreover, the synthesized κCa GO is capable of demulsification with an average demulsification efficiency of 70%. It was found that the demulsification efficiency increases with salinity and κCa GO dosage, and it deteriorates under alkaline condition. These phenomena can be attributed to the interfacial interactions between κCa GO and the emulsion. Furthermore, the κCa GO can be recycled to use for up to six cycles without significant leaching and degradation. As such, the synthesized κCa GO could be further developed as a potential sorbent substitute for the separation of crude oil from produced water.  相似文献   
60.
DNA methylation is a prevalent regulatory modification in prokaryotes and eukaryotes. N1-methyladenine (m1A) and N6-methyladenine (m6A) have been found to be capable of altering DNA structures via disturbing Watson–Crick base pairing. However, little has been known about their influences on non-B DNA structures, which are associated with genetic instabilities. In this work, we investigated the effects of m1A and m6A on both the structure and thermodynamic stability of a newly reported DNA minidumbbell formed by two TTTA tetranucleotide repeats. As revealed by the results of nuclear magnetic resonance spectroscopic studies, both m1A and m6A favored the formation of a T·m1A and T·m6A Hoogsteen base pair, respectively. More intriguingly, the m1A and m6A modifications brought about stabilization and destabilization effects on the DNA minidumbbell, respectively. This work provides new biophysical insights into the effects of adenine methylation on the structure and thermodynamic stability of DNA.  相似文献   
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