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101.
The pathology of aerosolized staphylococcal enterotoxin B (SEB) was studied in the nonhuman primate. Six juvenile rhesus monkeys that received multiple lethal inhaled doses of SEB developed diarrhea and vomiting within 24 hr followed by depression, dyspnea, and shock. Three of 6 animals died by 52 hr. The most striking gross lesion in all 6 monkeys was diffuse severe pulmonary edema. Histologically, edema fluid was present within the peribronchiolar, peribronchial, and perivascular interstitium, alveolar septa, and alveoli. The adventitia of pulmonary vessels was infiltrated by lymphocytes, macrophages, and fewer neutrophils. Numerous large lymphocytes with occasional mitotic figures were within pulmonary vessels, often occluding alveolar capillaries. These cells were strongly immunoreactive with monoclonal antibodies against CD3, establishing them as T cells. Ultrastructurally, endothelial cell junctions were intact, and endothelial cells and type I pneumocytes contained numerous pinocytotic vesicles. Alveolar septal interstitial spaces were expanded by edema. The mechanism of these SEB-induced pulmonary lesions was not determined. We hypothesize that cytokine production by activated T cells may have caused vascular permeability changes leading to widespread pulmonary edema and shock.  相似文献   
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BACKGROUND AND AIMS: Uroporphyrin and protoporphyrin produce alterations on 5-aminolevulinic acid dehydratase and porphobilinogen deaminase, as a result of a direct effect of porphyrins on the protein structure. With the aim of assessing the possible protection from the porphyrins effect on the proteins, some chemicals and the enzyme substrates were assayed. METHODS: Enzymes were pre-incubated with the protecting agents (beta-mercaptoethanol, dithiotreitol, hydroxylamine, succinic anhydride) or the corresponding substrates (delta-aminolevulinic acid and porphobilinogen), and then exposed to the porphyrins. All experiments were performed in the enzyme solutions after removing the porphyrins. RESULTS: The presence of sulfhydryl reagents partially protected both the enzyme activities and the content of total SH and free amino groups, but they did not prevent the appearance of molecular aggregates in the electrophoresis. Similar results were obtained in the presence of the corresponding substrates. Nucleophilic addition of hydroxylamine to the aromatic amino acids on the enzymes and blockage of their free amino groups did not prevent the direct effect of porphyrins, but these agents protected the enzyme activities from the photodynamic action of the tetrapyrroles, and also prevented the formation of molecular aggregates. However, an increased amount of free amino groups was observed, probably due to protein fragmentation. CONCLUSIONS: Porphyrins mainly affected the SH groups at or near the active site of the enzymes. Most of the free amino groups on the treated enzymes were involved in the formation of cross-links among the protein molecules. Protein fragmentation induced by porphyrins under UV light, and the consequent increased amount of free amino groups, were observed.  相似文献   
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We report the cosegregation of an arginine to leucine substitution at position 121 of the Norrie disease protein in a large kindred where exudative vitreoretinopathy segregates as an X-linked recessive trait. The clinical phenotype and rate of disease progression were extremely variable, with progression to total retinal detachment from less than age 2 years to more than 21 years. To date, all mutations in X-linked vitreoretinopathy have been missense mutations, presumably not affecting the three-dimensional structure of the NDP gene product, and clustered around residues 121-126 of the Norrie protein. This contrasts with the diversity of mutations seen in the more severe, allelic Norrie disease.  相似文献   
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A random sample (N = 197) of supported employees with mental retardation was examined in a longitudinal study. Results indicate that intelligence, prior earnings, and federal job subsidy predicted future earnings. Statistical controls applied to the stratified sample show that job placement, job type, subsidy, and means of transportation had little influence on earnings.  相似文献   
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ADP-ribosylation factor 5 (ARF5) is a member of the ARF gene family. The ARF proteins stimulate the in vitro ADP-ribosyltransferase activity of cholera toxin and appear to play a role in vesicular trafficking in vivo. We have mapped ARF5, one of the six known mammalian ARF genes, to a well-defined yeast artificial chromosome contig on human chromosome 7q31.3. In addition, we have isolated and sequenced an approximately 3.2-kb genomic segment that contains the entire ARF5 coding region, revealing the complete intron-exon structure of the gene. With six coding exons and five introns, the genomic structure of ARF5 is unique among the mammalian ARF genes and provides insight about the evolutionary history of this ancient gene family.  相似文献   
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