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31.
Unstable expansion of the CTG repeats in the 3' untranslated region encoding a member of the protein kinase family in the q13.3 band on chromosome 19 is a mutation specific for myotonic dystrophy. To examine the correlation between clinical expression and CTG trinucleotide repeat length, we carried out Southern blot analysis in a family with myotonic dystrophy. In this pedigree, the expanded CTG repeats were transmitted maternally. The mother had three female children. The mother had about 200 CTG repeats, and the number of repeats for each child was about 800, 1500 and 1600 in birth order. The mother and the patient with 800 repeats were unaware of muscle weakness or myotonia. Symptoms were present from age 3 years in the patient with 1500 repeats and from birth in the one with 1600 repeats. Although the mother menstruated regularly, the patients with 800 and 1500 repeats both menstruated irregularly, and the one with 1600 repeats has never menstruated. The age of onset and severity of the disease were correlated with the size of the expanded repeats. Endocrinological studies revealed that the basal levels of the gonadotropins, PRL and E2 were within normal range, and a pituitary response to LHRH was observed. These data suggest that the amenorrhea and menstrual irregularities were caused by a suprahypophyseal dysfunction. When expanded CTG repeats are transmitted maternally, abnormal products resulting from the metabolic disturbance in the affected mother may harm the fetus in utero. A heterozygous fetus, who has more CTG repeats, may be unable to metabolize the pathologic products sufficiently and therefore may become more severely affected. This may explain the exclusive maternal transmission of congenital myotonic dystrophy.  相似文献   
32.
Urinary protein and calcium excretion were assessed in 77 patients with the hepatic glycogen storage diseases (GSD): 30 with GSD-I (median age 12.4 years, range 3.2-32.9 years), 25 with GSD-III (median age 10.5 years, range 4.2-31.3 years) and 22 with GSD-IX (median age 11.8 years, range 1.2-35.4 years). Inulin (Cinulin) and para-aminohippuric acid (CPAH) clearances were also measured in 33 of these patients. Those with GSD-I had significantly greater albumin (F = 15.07, P < 0.001), retinol-binding protein (RBP) (F = 14.66, P < 0.001), N-acetyl-beta-D-glucosaminidase (NAG) (F = 9.41, P < 0.001) and calcium (F = 7.41, P = 0.001) excretion than those with GSD-III and GSD-IX. GSD-I patients (n = 18) also had significantly higher Cinulin (F = 5.57, P = 0.009), but CPAH did not differ (F = 0.77, NS). Renal function was normal in GSD-III and GSD-IX patients. In GSD-I, Cinulin (r = -0.51, P = 0.03) and NAG excretion (r = -0.40, P = 0.03) were inversely correlated with age, whereas albumin excretion was positively correlated with age (r = +0.41, P = 0.03). RBP and calcium excretion were generally high throughout all age groups. Hyperfiltration in GSD-I is associated with renal tubular proteinuria that occurs before the onset of significant albuminuria. Deficiency of glucose-6-phosphatase within the proximal renal tubule may primarily cause tubular dysfunction, glomerular hyperfiltration being a secondary phenomenon.  相似文献   
33.
An investigation of pore cracking in titanium welds   总被引:1,自引:0,他引:1  
Two welded Ti-6A1-4V pressure vessels leaked prematurely in service. The leaks were caused by cracks emanating from weld porosity. The cracks originated during fabrication, with subsequent growth in service leading to the formation of the leak paths. Pore cracking is thought to be caused by a mechanism that involves both sustained-load and cyclic contributions, with the former being the more prominent. It is shown that the tendency for cracking is influenced by pore position and that pore size is not a deciding factor in that regard. The factors that govern pore cracking are discussed, and the possible role of interstitial embrittlement is assessed.  相似文献   
34.
We address the issue of tracking moving objects in an environment covered by multiple uncalibrated cameras with overlapping fields of view, typical of most surveillance setups. In such a scenario, it is essential to establish correspondence between tracks of the same object, seen in different cameras, to recover complete information about the object. We call this the problem of consistent labeling of objects when seen in multiple cameras. We employ a novel approach of finding the limits of field of view (FOV) of each camera as visible in the other cameras. We show that, if the FOV lines are known, it is possible to disambiguate between multiple possibilities for correspondence. We present a method to automatically recover these lines by observing motion in the environment, Furthermore, once these lines are initialized, the homography between the views can also be recovered. We present results on indoor and outdoor sequences containing persons and vehicles.  相似文献   
35.
Precipitation of nickel ammonium sulphate achieved by mixing solutions of nickel sulphate and ammonium sulphate in a 5 1 MSMPR crystallizer is used to investigate both crystallization and agglomeration kinetics from the steady-state crystal size distribution. An iterative non-linear parameter estimation procedure is used to deduce the kinetic rate parameters in the solution of the agglomeration model suggested by Liao and Hulburt from the data-set obtained by size analysis of the product crystals. The results obtained are correlated in terms of conventional power law kinetic expressions.  相似文献   
36.
The molecular weight of polyacrylic acid (PAA) was determined by a viscometric method using NaNO3 as solvent at 30°C. The specific electric conductivities (σ) of PAA as well as PAA doped with carbon black (CB), chromium oxide (Cr2O3), and cupferron with different concentrations (from 0.25 to 1 wt %) were measured at a temperature range 360–400 K. IR spectra of some polymers were determined and it was shown that when PAA was doped with 0.5 wt % CB, a C? O? C band appeared at 775–875 cm?1. The positron annihilation lifetime (PAL) spectra in PAA doped with the above‐mentioned dopants were measured as a function of their concentrations. It was observed that the short lifetime intensity I1 decreased, whereas the intermediate lifetime intensity I2, which is related to the conductivity of the material, increased with increasing the wt % of Cr2O3 and cupferron as well as at low concentrations of CB. These results are discussed in terms of the conducting island model. It was found that there were distinct positive relationships between σ and I2. © 2002 John Wiley & Sons, Inc. J Appl Polym Sci 84: 877–883, 2002; DOI 10.1002/app.10381  相似文献   
37.
It is conjectured that anionic-cationic surfactant combination can be regarded as equivalent to a double chain surfactant and using molecular packing considerations it is shown that vesicles, viscoelastic solutions and liquid crystals can be designed by the proper choice of chain lengths of the pair. Using these concepts new systems are designed, from mixtures of cetyltrimethyl ammonium bromide and sodium alkyl sulfonates, to produce both viscoelastic gels and vesicles.  相似文献   
38.
Geometry-Dependent R-Curve for Quasi-Brittle Materials   总被引:4,自引:0,他引:4  
Fracture of quasi-brittle materials such as ceramics and cement-based materials can be described by an R -curve, defined as fracture resistance. The R -curve can be constructed as the envelope of the strain energy release rates ( G ). In this study, R -curve is defined as an envelope of G -curves obtained by varying the dimensions of identical specimens, while keeping the geometry and the initial flaw size constant. By approximating the G -curve with a second-order function of the crack length, a simple formulation of the R -curve is derived by solving a differential equation. Three parameters are needed for the proposed R -curve. These parameters can be obtained by testing a notched-beam specimen and using linear elastic fracture mechanics. The proposed R -curve simulates well the geometry dependency as well as other characteristics of the fracture response of quasi-brittle materials.  相似文献   
39.
A routing protocol chooses one of the several paths (routes) from a source node to a destination node in the computer network, to send a packet of information. In this paper, we propose a new routing protocol, which we call st-routing protocol, based on st-numbering of a graph. The protocol fits well in noisy environments where robustness of routing using alternative paths is a major issue. The proposed routing protocol provides a systematic way to retry alternative paths without generating any duplicate packets. The protocol works for only those networks that can be represented by biconnected graphs.  相似文献   
40.
Continued research into the development of III-V high-electron mobility transistors (HEMTs), specifically the minimization of the device gate length, has yielded the fastest performance reported for any three terminal devices to date. In addition, more recent research has begun to focus on reducing the parasitic device elements such as access resistance and gate fringing capacitance, which become crucial for short gate length device performance maximization. Adopting a self-aligned T-gate architecture is one method used to reduce parasitic device access resistance, but at the cost of increasing parasitic gate fringing capacitances. As the device gate length is then reduced, the benefits of the self-aligned gate process come into question, as at these ultrashort-gate dimensions, the magnitude of the static fringing capacitances will have a greater impact on performance. To better understand the influence of these issues on the dc and RF performance of short gate length InP pHEMTs, the authors present a comparison between In0.7Ga0.3As channel 50-nm self-aligned and "standard" T-gate devices. Figures of merit for these devices include transconductance greater than 1.9 S/mm, drive current in the range 1.4 A/mm, and fT up to 490 GHz. Simulation of the parasitic capacitances associated with the self-aligned gate structure then leads a discussion concerning the realistic benefits of incorporating the self-aligned gate process into a sub-50-nm HEMT system  相似文献   
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