Advances on the polycystic liver diseases
-
摘要: 多囊肝病(PCLD)是种遗传病,病因复杂,表型表现多样。按遗传方式其又分为常染色体显性遗传PCLD和常染色体隐性遗传PCLD。PCLD囊肿上皮初级纤毛异常及其对多种生长因子增殖效应的敏感性,为将来治疗提供了靶位。Abstract: Polycystic liver diseases (PCLDs) are genetic disorders with heterogeneous etiologies and a range of phenotypic presentations.PCLD exhibits both autosomal or recessive dominant pattern of inheritance.The abnormalities of primary cilia and the sensitivity to proliferative effects of different growth factors in PCLD cystic epithelium provide the basis for future treatment targets.
-
Key words:
- liver diseases /
- cysts
-
[1]Everson GT, Helmke SM, Doctor B.Advances inmanagement of polycystic liver disease[J].Expert RevGastroenterol Hepatol, 2008, 2 (4) :563-576. [2]Shneider BL, Magid MS.Liver disease in autosomal recessivepolycystic kidney disease[J].Pediatr Transpl, 2005, 9 (5) :634-639. [3]Torres VE, Harris PC.Autosomal dominant polycystic kidneydisease:the last 3 years[J].Kidney Int, 2009, 76 (2) :149-168. [4]Masyuk T, Masyuk A, LaRusso N.Cholangiociliopathies:genetics, molecular mechanisms and potential therapies[J].Curr Opin Gastroenterol, 2009, 25 (3) :265-271. [5]Fencl F, Janda J, Blahova K, et al.Genotype-phenotypecorrelation in children with autosomal dominant polycystickidney disease[J].Pediatr Nephrol, 2009, 24 (5) :983-989. [6]江雨, 周裕林.常染色体隐性遗传性多囊肾病 (ARPKD) 基因缺陷的研究进展[J].中国优生与遗传学杂志, 2007, 15 (2) :114-115. [7]Masyuk AI, Masyuk TV, LaRusso NF.Cholangiocyteprimary cilia in liver health and disease[J].Dev Dyn, 2008, 237 (8) :2007-2012. [8]Torrice A, Cardinale V, Gatto M, et al.Polycystins play a keyrole in the modulation of cholangiocyte proliferation[J].DigLiver Dis, 2010, 42 (5) :377-385. [9]Muchatuta MN, Gattone VH 2nd, Witzmann FA, et al.Structural and functional analyses of liver cysts from theBALB/c-cpk mouse model of polycystic kidney disease[J].Exp Biol Med (Maywood) , 2009, 234 (1) :17-27. [10]Alvaro D, Onori P, Alpini G, et al.Morphological andfunctional features of hepatic cyst epithelium in autosomaldominant polycystic kidney disease[J].Am J Pathol, 2008, 172 (2) :321-332. [11]Nishio S, Hatano M, Nagata M, et al.Pkd1 regulatesimmortalized proliferation of renal tubular epithelial cellsthrough p53 induction and JNK activation[J].J Clin Invest, 2005, 115 (4) :910-918. [12]Brodsky KS, McWilliams RR, Amura CR, et al.Livercyst cytokines promote endothelial cell proliferationand development[J].Exp Biol Med (Maywood) , 2009, 234 (10) :1155-1165. [13]Fabris L, Cadamuro M, Fiorotto R, et al.Effects of angiogenicfactor overexpression by human and rodent cholangiocytesin polycystic liver diseases[J].Hepatology, 2006, 43 (5) :1001-1012. [14]Spirli C, Okolicsanyi S, Fiorotto R, et al.ERK1/2-dependentvascular endothelial growth factor signaling sustains cystgrowth in polycystin-2 defective mice[J].Gastroenterology, 2010, 138 (1) :360-371. [15]Glaser SS, Gaudio E, Miller T, et al.Cholangiocyteproliferation and liver fibrosis[J].Expert Rev Mol Med, 2009, 11:e7. [16]Keimpema LV, Nevens F, Vanslembrouck R, et al.Lanreotidereduces the volume of polycystic liver:A randomized, double-blind, placebo-controlled trial[J].Gastroenterology, 2009, 137 (5) :1661-1668. [17]Wang S, Zhang J, Nauli SM, et al.Fibrocystin/polyductin, found in the same protein complex with polycystin-2, regulates calcium responses in kidney epithelia[J].Mol CellBiol, 2007, 27 (8) :3241-3252. [18]Banales JM, Masyuk TV, Gradilone SA, et al.The cAMPeffectors Epac and protein kinase a (PKA) are involved inthe hepatic cystogenesis of an animal model of autosomalrecessive polycystic kidney disease (ARPKD) [J].Hepatology, 2009, 49 (1) :160-174. [19]Amura CR, Brodsky KS, Gitomer B, et al.CXCR2 agonistsin ADPKD liver cyst fluids promote cell proliferation[J].Am JPhysiol Cell Physiol, 2008, 294 (3) :C786-C796. [20]Lai X, Blazer-Yost BL, Gattone VH 2nd, et al.Proteincomposition of liver cyst fluid from the BALB/c-cpk/+mousemodel of autosomal recessive polycystic kidney disease[J].Proteomics, 2009, 9 (14) :3775-3782. [21]Masyuk T, Masyuk A, LaRusso N.MicroRNAs incholangiociliopathies[J].Cell Cycle, 2009, 8 (9) :1324-1328.
本文二维码
计量
- 文章访问数: 3011
- HTML全文浏览量: 17
- PDF下载量: 1984
- 被引次数: 0