Recombinant Mouse Polycystin-1 (Pkd1), partial

1. Results indicate that PKD1 inactivation underlies excitotoxicity-induced neuronal death and suggest that PKD1 inactivation may be critical for the accumulation of oxidation-induced neuronal damage during aging and in neurodegenerative disorders. PMID: 29273751
2. PKD1 contributes to the osteoblast differentiation and bone development via elevation of osteoblast markers through activation of STAT3 and p38 MAPK signaling pathways. PMID: 28084409
3. PC1/3 deficiency was associated with increased expression of melanocortin receptors and PRCP (prolylcarboxypeptidase, a catabolic enzyme for alpha-melanocyte stimulating hormone (alphaMSH)), and reduced adrenocorticotropic hormone secretion. We conclude that the obesity accompanying PCSK1 deficiency may not be primarily due to alphaMSH deficiency. PMID: 28132887
4. Mutations in PKD1 is associated with autosomal dominant polycystic kidney disease. PMID: 28007903
5. critical functions of PC1 are regulated by its ability to sense cytosolic calcium levels via binding to calmodulin PMID: 27560828
6. cortactin binds to E-cadherin, and that a posttranslational modification of cortactin, RhoA-induced phosphorylation by protein kinase D1 (PKD1; also known as PRKD1) at S298, impairs adherens junction assembly and supports their dissolution. PMID: 27179075
7. Galpha12 is required for the development of kidney cysts induced by Pkd1 mutation in mouse autosomal dominant polycystic kidney disease. PMID: 27505895
8. kd1 mutant mice have transcriptional profiles consistent with changes in lipid metabolism and distinct metabolite and complex lipid profiles in kidneys. .. cells lacking Pkd1 have an intrinsic fatty acid oxidation defect and that manipulation of lipid content of mouse chow modifies cystic disease. PMID: 27077126
9. Polycystin 1 was overexpressed in M1 cells, no increase in any of these parameters was detected PMID: 26741910
10. detected a marked increase in the localization of beta-catenin in the nucleus of crypt epithelial cells in the ileum of PKD1 PMID: 26739494
11. PKD1 phosphorylates AMPKalpha2 at Ser485/491, thus diminishing AMPK activity. PMID: 26797128
12. These data potentially explain the severe renal manifestations of the tuberous sclerosis/polycystic kidney disease contiguous gene syndrome and open new perspectives for the use of mTOR inhibitors in PKD. PMID: 26931735
13. novel protein complex composed of Rabep1, GGA1 and Arl3 is responsible for the sorting and targeting of the polycystin 1 andpolycystin 2 to the cilium. PMID: 25405894
14. Polycystin-1 regulation of the microtubule cytoskeleton impacts on the turnover rates of focal adhesions in migrating cells and we link all these properties to the capability of PC-1 to regulate the activation state of Focal Adhesion Kinase. PMID: 25947155
15. Pkd1 and pkd2 are present in primary cilia of radial glia. Ablation of Pkd1 or Pkd2 affected planar cell polarity development in radial glia and epithelial cells. PMID: 26245976
16. Pkd1 haploinsufficiency accelerated the development of tubular dilations after nephron reduction, a phenotype that was associated to a further increase of cell proliferation in a model of polycystic kidney disease. PMID: 25531116
17. Pkd1 mutation or deletion leads to the activation of Galpha12, which promotes the maturation of ADAM10 that increases the shedding of E-cadherin in kidney epithelial cells. PMID: 25492927
18. PC-1 knockout mice manifested decreased cardiac function relative to littermate controls, and alpha1C L-type calcium channel protein levels were significantly lower in PC-1 knockout hearts. PMID: 25888683
19. PKD1 gene deletion is causing disease progression in polycystic kidney disease in animal models. PMID: 25361818
20. The results of this study demonstrate that PC1 trafficking and expression require GPS cleavage and PC2 interaction, respectively, and provide a framework for functional assays to categorize the effects of missense mutations in polycystins. PMID: 25365220
21. Pkd1 deleted in the principal cells led to progressive cystic kidney disease, while Pkd1 deleted in the intercalated cells had a very mild cystic phenotype PMID: 19145237
22. PKD1 is a key regulator involved in determining the threshold at which mitochondrial membranes depolarize after reactive oxygen-induced stress. PMID: 25759386
23. Results suggest that PC1 is a BBSome cargo and that the components of the BBSome may possess subunit-specific functions. PMID: 24939912
24. Polycystin-1 mediates mechanical strain-induced osteoblastic mechanoresponses via potentiation of intracellular calcium and Akt/beta-catenin pathway. PMID: 24618832
25. PC2 regulates PC1 maturation; therefore, mature PC1 levels are a determinant of disease severity in PKD2 as well as PKD1. PMID: 25574838
26. Pkd1(RC/RC) mice experience hepatic hypertrophy that can be corrected by pasireotide through a pathway involving AC6 and cAMP PMID: 24994926
27. Our data reveal for the first time differences between TRPP1 and TRPP2 deficiency PMID: 24193408
28. Cyst growth most probably plays a central role in early-stage ADPKD-associated hypertension in Pkd1-deficient mice. PMID: 24429399
29. found a complex pattern of endogenous Pc1 forms by cleavage at the G protein-coupled receptor proteolytic site PMID: 24958103
30. Loss of Pkd1 in mice has no effect on precursor sprouting but leads to failed morphogenesis of the subcutaneous lymphatic network. PMID: 24767999
31. discovered that loss of the Pkd1 gene product, polycystin-1 (PC1), whose mutation causes human autosomal dominant polycystic kidney disease (ADPKD), downregulates Bicc1 expression in vitro and in vivo PMID: 24594709
32. activation of the signaling effectors Src, Erk, and the mTOR effector S6 was enhanced, and Ca(2+) response to external stimuli was reduced, in Pkd1(-/-);Nedd9(-/-) versus Pkd1(-/-) mice. PMID: 25139996
33. Depletion of SIRT2 prevented the abnormal centrosome amplification and polyploidy associated with loss of polycystin-1 (PC1) alone. PMID: 24203696
34. Expression of PC1 and PC2 in osteocyte-like cells is increased by oscillatory fluid flow PMID: 24268313
35. this uncovered a new pathway suggesting that when PC1 is expressed, PC2 that is not bound to PC1 is directed to aggresomes and subsequently degraded via autophagy, a control mechanism that may play a role in autosomal dominant polycystic kidney disease pathogenesis. PMID: 24459142
36. PKD1 mediates negative feedback of PI3K/Akt activation in response to G protein-coupled receptors. PMID: 24039875
37. Pkd1, whose mutations account for 85% of polycystic kidney, autosomal dominant cases in humans, is essential for male reproductive tract development. PMID: 23933588
38. Glomeruli fail to properly develop in Pkd1 mutant renal organ cultures. Defective endothelial cell migration likely accounts for glomerulogenesis defects. PMID: 24472557
39. We targeted mutations in Pkd1 and Fbn1. Double heterozygotes displayed an exacerbation of the typical Fbn1 heterozygous aortic phenotype. The basis of this genetic interaction results from upregulation of TGF-beta signaling caused by Pkd1 haploinsufficiency. PMID: 24071006
40. Overexpression of PC-1 C-tail in M-1 C20 cells is associated with an ouabain-sensitive phenotype and an increased ability of the cells to proliferate and secrete anions upon ouabain stimulation. PMID: 23784065
41. These results reveal a reciprocal functional link between PC1 and PC2 which is critically dependent on their interaction. PMID: 23390129
42. The Pkd1/Hnf4alpha double mutants have significantly more cystic kidneys, thus indicating that metabolic pathways could play a role in Pkd1-cystogenesis PMID: 23209428
43. Rapamycin treatment reduces cyst formation in Pkd1(-/-) mutant mice; therefore, the prevention of kidney cyst expansion in utero by mTOR inhibition is feasible. PMID: 22725947
44. Deletion of Pkd1 in mesenchymal precursors resulted in pancreatic and renal, but not hepatic, cyst formation. PMID: 23029375
45. miR-200b/c/429 induce post-transcriptional repression of Pkd1 through two conserved binding sites in the 3'-Untranslated regions of Pkd1 PMID: 23138483
46. Aberrant p38delta-PKD1 signaling in neutrophils may underlie development of acute lung injury and life-threatening acute respiratory distress syndrome. PMID: 23129748
47. Functional polycystin-1 dosage governs autosomal dominant polycystic kidney disease severity. PMID: 23064367
48. Polycystin-1 forms a holoenzyme complex with PP1alpha via a conserved PP1-binding motif. PMID: 22675472
49. Proteolytic events that remove the C1 domain (but not the autoinhibitory PH domain) limit maximal PKD1 activity toward physiologically relevant protein substrates and lead to a defect in PKD1-dependent cellular responses. PMID: 22582392
50. Centrosome amplification occurs in kidneys from conditional Pkd1 knockout mice and in human autosomal dominant polycystic kidney disease patients. PMID: 18566106

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KEGG: mmu:18763

STRING: 10090.ENSMUSP00000049296

UniGene: Mm.290442