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1.
In the past 5 years we have discovered 8 boys and 3 girls who suffer from fructose-1,6-bisphosphatase deficiency. Although they all showed the typical symptoms of the deficiency such as frequent vomiting, hypoglycemia, lactic acidosis, and hepatomegaly, the age at diagnosis varied from 2 months to 4 years. All the boys revealed the deficient enzyme activity in leukocytes but none of the girls. The liver biopsy was investigated in six patients to confirm the diagnosis. These results suggest the existence of tissue-specific isoenzymes for fructose-1,6-bisphosphatase possibly with a different gene origin.  相似文献   
2.
Current cementing techniques of distal plugging, pulsatile water cleaning of the canal, and retrograde filling with PMMA in a low-viscous state prepared using porosity reduction techniques and then pressurized have been shown to give excellent results at 10 or more years after operation. However, physical and aging characteristics of PMMA do not guarantee that those results will hold up in the 20- to 30-year time frame. Fifteen-year experience with bony ingrowth systems indicate the development of a durable interface without PMMA, although the interface does appear more vulnerable to attack by the biologic reaction to HMWPE debris. Bone remodeling appears more favorable around proximally fixed bone ingrowth prostheses than around distally fixed prostheses. Softening of the physical characteristics of the stem tip may reduce the incidence of thigh pain. Patients with high activity potential and a life expectancy of greater than 25 years should be strongly considered for a proximally fixed bone ingrowth or ongrowth prosthesis.  相似文献   
3.
Elements of the lactose operon were used to study parameters affecting gene expression in cultured cells and transgenic animals. A Lac repressor protein containing a nuclear transport signal was shown to inhibit expression of a reporter gene by interacting with lac operator sequences. In cultured cells, operator sequence, operator placement and induction parameters were all shown to be important for obtaining tight repression of a reporter gene followed by high level expression upon induction. Induction levels were also dependent on the reporter gene, with the luciferase gene yielding higher induction levels than the chloramphenicol acetyltransferase gene. In transgenic animals, the lacI mRNA was not detected in the C57BL/6 mouse strain until the animal was exposed to a demethylating agent. After 5-azacytidine treatment, expression of lacI mRNA was detected in the brain, heart, kidney, lung and ovary. In the FVB transgenic mouse strain, expression of lacI mRNA was detected without 5-azacytidine treatment in the kidney, liver, lung, and testes. Preliminary experiments with double transgenic animals containing both lacI and operator/luciferase transgenes showed a decrease in luciferase expression compared to the luciferase-only animals in both tissue extracts and transgenic fetal primary cultures, although IPTG induction was not achieved in these animals or primary cultures. The applicability and challenges of the system for regulation of gene expression are discussed.  相似文献   
4.
Complexation of chitosan in aqueous solutions by low molecular weight electrolytes is one of the simplest methods for the preparation of aqueous chitosan dispersions. In this work, the influence of storage time, sulfate concentration, method of preparation and surfactant content on some properties of the resultant chitosan dispersions (turbidity, viscosity and zeta potential) was analyzed. Turbidimetry was adequate to monitor the formation of particles, while viscometry was suitable to monitor changes in the dispersing phase. An analysis of the properties of these systems, mainly in terms of particle–particle and macromolecule–macromolecule interactions was carried out. Copyright © 2004 Society of Chemical Industry  相似文献   
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6.
Heart transplant patients take several medications that could affect their periodontal health. Gingival overgrowth associated with cyclosporin (immunosuppressant agent) and nifedipine (calcium channel blocker) is well documented. Candidal infections often develop because of immune suppression. This report describes the clinical and histopathological changes in the gingival tissues of a heart transplant patient and their management. The gingival tissues exhibited pronounced enlargement. The gingivae were lobulated, and the surface of the lobulations was pebbly and granular. Biopsies showed lobules of fibrous connective tissue covered by stratified squamous epithelium. The outer surfaces were dotted with numerous smaller papillations. Candidal hyphae were present in the superficial layers of the epithelium. The extensive papillary lesions appear to be related to candidiasis and constitute a condition which is best designated as papillary stomatitis. Hyperplastic gingival tissues were excised, and the patient was placed on periodic maintenance. One-year postoperative follow-up showed minor gingival growth.  相似文献   
7.
The interaction of monovalent Fab fragments of NC10, an antiviral neuraminidase antibody, and the anti-idiotype antibody 3-2G12 has been used as a model system to demonstrate experimentally the influence of non-ideal binding effects on BIAcore binding data. Because the association rate constant for these two molecules was found to be relatively high (about 5 x 10(5) M-1 S-1), mass transfer was recognised as a potential source of error in the analysis of the interaction kinetics. By manipulation of the flow rate and the surface density of the immobilised ligand, however, the magnitude to this error was minimised. In addition, the application of site-specific immobilisation procedures was found to improve considerably the correlation of experimental binding data to the ideal 1:1 kinetic model such that the discrepancy between experimental and fitted curves was within the noise range of the instrument. Experiments performed to measure the equilibrium constant (KD) in solution resulted in a value of similar magnitude to those obtained from the ratio of the kinetic rate constants, even those measured with a heterogeneous ligand or with a significant mass transfer component. For this system, the experimental complexities introduced by covalent immobilisation did not lead to large errors in the KD values obtained using the BIAcore.  相似文献   
8.
An animal model and computer software were developed to conduct across-herd genetic evaluations using data from producers participating in the Sow Productivity Index program of the American Yorkshire Club. The final data set consisted of 61,596 litter records from 1986 to early 1990. The animal model included fixed contemporary group effects and random additive direct, service sire, permanent environmental, and residual effects. Additive genetic relationships among animals were included. A separate relationship matrix for service sires and their sires was also included. A data set similar to the Yorkshire field data was simulated to use in testing the animal model. The simulated data set consisted of 40 herds, each with 120 reproducing dams and either four or five sires. Six generations of simulated data were produced, resulting in 20,605 litter records. These records were then evaluated using the animal model for number of pigs born alive. Finally, correlations between the true breeding values from the simulation and the predicted breeding values were computed. The correlation between the 918 true and predicted sire breeding values was considerably lower for the animal model without a service sire effect than when it was included (.53 vs .74, respectively). However, the difference was cut in half (.66 vs .77) when only sires with greater than five daughter records were included. The high accuracy of the animal model with a random service sire effect indicates that the proposed model adequately accounts for the variation found in records for number of pigs born alive.  相似文献   
9.
We examined the ability of opsonized zymosan (OPZ) to stimulate translocation of protein kinase C (PKC) isoforms in human neutrophils. Neutrophils express five PKC isoforms (alpha, betaI, betaII, delta, and zeta), but little is known of their individual roles in neutrophil activation. As determined by immunoblotting, OPZ caused a time-dependent translocation of the predominant PKC isoforms (betaII, delta, and zeta) to neutrophil membranes, with a concomitant loss from the cytosol. Maximal translocation of all three isoforms occurred by 3 min. No PKC immunoreactivity was observed in a crude nuclear fraction, but PKC-delta and -zeta were found in the granule fraction after degranulation (10 min). PKC activity (Ca2+-dependent and -independent) increased 50- and 19-fold, respectively, by 10 min in the granules from OPZ-stimulated cells. Curiously, no immunoreactive cPKC (alpha and beta(I/II)) could be localized in the granule fraction to account for the Ca2+-dependent PKC activity. Localization of PKC isoforms in the neutrophil membranes and granules suggests their involvement in the regulation of functional responses triggered by OPZ. PKC isoform translocation to membranes from OPZ-stimulated cells preceded both p47phox (a cytosolic component of the NADPH oxidase) translocation and NADPH oxidase assembly. The presence of both PKC isoforms and p47phox in the membrane was transient, with the loss of p47phox occurring sooner than either the loss of membrane-associated PKC or that of NADPH oxidase activity. The apparent EC50 values for PKC translocation and NADPH oxidase assembly were similar. These data suggest that PKC isoforms regulate the assembly and activation of NADPH oxidase induced by OPZ.  相似文献   
10.
We have used the fluorescence in situ hybridization (FISH) technique to refine the localization of the cystic fibrosis transmembrane conductance regulator (CFTR) gene on human chromosome 7. The result shows that the gene is most likely located within band q31.3.  相似文献   
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