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231.
Deficiency of amylo-1,6–glucosidase activity was expressed in parallel in liver and skin fibroblasts from a patient with type III glycogenosis. In crude extracts of control liver and muscle, amylo-1, 6–glucosidase (M.W. 164000) was identified by immunoprecipitation; no cross-reacting material was found in the patient's liver. Assay of amylo-1,6–glucosidase activity in cultured skin fibroblasts from the affected family revealed less than 10 per cent of control value in mutant homozygous cells whereas in cells from the parents, activity was reduced to 40–60 per cent of the control value. Activity in cultured amniotic fluid cells was similar to that of control fibroblasts. In cultured amniotic fluid cells obtained during the mother's subsequent pregnancy, the normal amylo–1,6–glucosidase activity measured, predicted correctly the outcome of this pregnancy prior to the 20th week of gestation.  相似文献   
232.
Trace metals were analyzed in the muscle and other organs from several species of deep-water sharks (particularly Centrophorus granulosus and Galeus melastomus) from 1280 to 1500 m depth in the eastern Mediterranean between 1985 and 1991. As has been shown with other pelagic fish, there was a significant correlation between specimen size and the mercury concentration in the muscle as well as in the liver and kidneys. For a given size, the level of mercury in sharks from the eastern Mediterranean was considerably higher compared with the same species caught off the west coast of Italy. There was no significant correlation between the concentrations of Cd, Cu, Zn and Fe in the muscle (or other organs) and body size. The overall trace metal content was considerably higher in the specimens from the eastern Mediterranean compared with the same species from the N. E. Atlantic. It is suggested that these higher-than-expected trace metal levels might be due to a relatively high trace metal content in the waters of the region or to the unusual physiology of these fish, possibly related to the extreme oligotrophic conditions in the area.  相似文献   
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INTRODUCTION: In Washington State, 87 workers are killed each year, on average, while in work status. To understand these incidents and to assist in focusing on and development of potential prevention measures, they must be well characterized. METHODS: Work-related fatalities between the years 1998 and 2002 are described by the demographics of the victims, types of incidents, the victims' occupations, and industries and location in which they worked. RESULTS: Motor vehicle- and machinery-related incidents accounted for nearly 33% and 14% of the incidents, respectively. Agriculture, forestry, fishing, hunting, and mining (n=87), and construction (n=83) had the most fatalities. Fatality rates per 100,000 workers for these industries were 25.7 and 8.7, respectively, compared to the state-wide average of 3.1 fatalities/100,000 workers. DISCUSSION: These data indicate numerous areas for prevention of work-related traumatic injuries and fatalities.  相似文献   
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