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Proposed mechanism for the turnover of myosin and its relation to muscular dystrophy 总被引:1,自引:0,他引:1
J F Aronson 《Nature》1966,210(5040):995-996
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W Bruening N Bardeesy B L Silverman R A Cohn G A Machin A J Aronson D Housman J Pelletier 《Nature genetics》1992,1(2):144-148
Denys-Drash syndrome is a rare human developmental disorder affecting the urogenital system and leading to renal failure, intersex disorders and Wilms' tumour. In this report, four individuals with this syndrome are described carrying germline point mutations in the Wilms' tumour suppressor gene, WT1. Three of these mutations were in the zinc finger domains of WT1. The fourth occurred within intron 9, preventing splicing at one of the alternatively chosen splice donor sites of exon 9 when assayed in vitro. These results provide genetic evidence for distinct functional roles of the WT1 isoforms in urogenital development. 相似文献
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Jiang Z Asplin JR Evan AP Rajendran VM Velazquez H Nottoli TP Binder HJ Aronson PS 《Nature genetics》2006,38(4):474-478
Urolithiasis is one of the most common urologic diseases in industrialized societies. Calcium oxalate is the predominant component in 70-80% of kidney stones, and small changes in urinary oxalate concentration affect the risk of stone formation. SLC26A6 is an anion exchanger expressed on the apical membrane in many epithelial tissues, including kidney and intestine. Among its transport activities, SLC26A6 mediates Cl(-)-oxalate exchange. Here we show that mutant mice lacking Slc26a6 develop a high incidence of calcium oxalate urolithiasis. Slc26a6-null mice have significant hyperoxaluria and elevation in plasma oxalate concentration that is greatly attenuated by dietary oxalate restriction. In vitro flux studies indicated that mice lacking Slc26a6 have a defect in intestinal oxalate secretion resulting in enhanced net absorption of oxalate. We conclude that the anion exchanger SLC26A6 has a major constitutive role in limiting net intestinal absorption of oxalate, thereby preventing hyperoxaluria and calcium oxalate urolithiasis. 相似文献
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Sporulation and δ-endotoxin synthesis by Bacillus thuringiensis 总被引:4,自引:0,他引:4
Aronson A 《Cellular and molecular life sciences : CMLS》2002,59(3):417-425
Bacillus thuringiensis is distinguished from the very closely related Bacillus cereus and Bacillus anthracis by the presence of several plasmid-encoded δ-endotoxin genes. These δ-endotoxins, synthesized as protoxins, are produced
in large quantities during sporulation and are packaged into intracellular inclusions. Ingestion of the inclusions by insect
larvae leads to protoxin solubilization and conversion to toxins each specific for one of several orders of insects. The toxins
form cation-selective channels in the membrane of cells lining the larval midgut with subsequent lethality. In most cases,
δ-endotoxin synthesis and sporulation are closely coupled. The latter process in B. thuringiensis is probably virtually identical to that in Bacillus subtilis with the additional use of mother cell sporulation forms of RNA polymerase for the synthesis of the δ-endotoxins. There are
other more subtle plasmid-encoded functions or plasmid interactions related to regulating protoxin synthesis. Consideration
of both plasmid and chromosomal genes is thus critical for defining this organism. 相似文献
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M. Kalina J. J. Bubis Y. Kletter A. Shahar M. Aronson 《Cellular and molecular life sciences : CMLS》1970,26(3):287-288
Résumé La localisation de la phosphatase-acide dans la levureCryptococcus neoformans est étudiée ici par des méthodes cytochimiques au niveau ultrastructural. On a trouvé que l'enzyme se concentrait exclusivement dans les vacuoles, ce qui s'accorde avec les résultats que d'autres auteurs ont obtenus sur la localisation de l'enzyme dans les cellules végétales. 相似文献
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