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1.
Autosomal dominant polycystic liver disease results from mutations in PRKCSH or SEC63. The respective gene products, glucosidase IIβ and SEC63p, function in protein translocation and quality control pathways in the endoplasmic reticulum. Here we show that glucosidase IIβ and Sec63p are required in mice for adequate expression of a functional complex of the polycystic kidney disease gene products, polycystin-1 and polycystin-2. We find that polycystin-1 is the rate-limiting component of this complex and that there is a dose-response relationship between cystic dilation and levels of functional polycystin-1 following mutation of Prkcsh or Sec63. Reduced expression of polycystin-1 also serves to sensitize the kidney to cyst formation resulting from mutations in Pkhd1, the recessive polycystic kidney disease gene. Finally, we show that proteasome inhibition increases steady-state levels of polycystin-1 in cells lacking glucosidase IIβ and that treatment with a proteasome inhibitor reduces cystic disease in orthologous gene models of human autosomal dominant polycystic liver disease.  相似文献   
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Summary A new norsesterterpene, hyrtial4, and known sesterterpenes,1–3, have been isolated from an anti-inflammatory active crude extract of the spongeHyrtios erecta.This sponge,Hyrtios (Heteronema) erecta Keller 1889, collected in 1980/81, from Tonga was extremely abundant in the Vava'u Is. Group.We thank Prof. R. Jacobs and his research group at UCSB for this data.  相似文献   
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Female mimicry in garter snakes   总被引:4,自引:0,他引:4  
R T Mason  D Crews 《Nature》1985,316(6023):59-60
In many diverse taxa, males of the same species often exhibit multiple mating strategies. One well-documented alternative male reproductive pattern is 'female mimicry', whereby males assume a female-like morphology or mimic female behaviour patterns. In some species males mimic both female morphology and behaviour. We report here female mimicry in a reptile, the red-sided garter snake (Thamnophis sirtalis parietalis). This form of mimicry is unique in that it is expressed as a physiological feminization. Courting male red-sided garter snakes detect a female-specific pheromone and normally avoid courting other males. However, a small proportion of males release a pheromone that attracts other males, as though they were females. In the field, mating aggregations of 5-17 males were observed formed around these individual attractive males, which we have termed 'she-males'. In competitive mating trails, she-males mated with females significantly more often than did normal males, demonstrating not only reproductive competence but also a possible selective advantage to males with this female-like pheromone.  相似文献   
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本文指出仪洪勋和Brosch G在具有三个判别的CM公共值的亚纯函数的唯一性定理中,关于对数函数的导数是整函数的推导,可以用指数函数求导的方法来证明.改进了仪洪勋和Brosch G关于重值与唯一性定理.  相似文献   
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Upon amputation of the urodele limb, the epidermal cells surrounding the amputation plane migrate to heal the wound. The resulting wound epidermis (WE) induces the regeneration process, resulting in blastema formation, cell division, and ultimately repatterning into a new limb. Despite its central role in the initiation of limb regeneration, little is known about how the WE forms. Here we discuss various models of WE formation and the experimental data in support of each.  相似文献   
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P Crews  P Bescansa  G J Bakus 《Experientia》1985,41(5):690-691
A new norsesterterpene, hyrtial 4, and known sesterterpenes, 1-3, have been isolated from an anti-inflammatory active crude extract of the sponge Hyrtios erecta.  相似文献   
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The bovine chromaffin cells (BCC) implanted into the subarachnoid space can release analgesic substances such as opioid peptides and ealeeholamines. Clinical trials have provided the evidence that the implantation of polyvinylchloride ( PVC) hollow fiber encapsulated BCC by surgery can relief the pain in cancer patients. In the present study, BCC were encapsulated in alginate-polylysine-alginate (APA) mieroencapsules which protect the grafting of xenogeneic cells from host immune system anil allow BCC to function effectively without using immunosuppression agents. The microencapsulated BCCs (5 X 106~—8 X 106) were transplanted into the subarachnoid space I^._s of 17 patients who suffered from chronic cancer pain and had to have long-term administration of analgesics. The pain scores and morphine intake tesl showed that microencapsulated BCC graft totally stopped the chronic pain in three of the patients over a period of 200 days and in the other three over a period of 100 days. The resulls suggesl thai APA microencapsulated BCC xenotransplantation could be a novel alternative approach to managing pain of cancer patients.  相似文献   
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