Genomes and Genes
Affiliation: University of Texas Southwestern Medical Center
- Proteolytic cleavage and nuclear translocation of fibrocystin is regulated by intracellular Ca2+ and activation of protein kinase CThomas Hiesberger
Department of Internal Medicine, University of Texas Southwestern Medical Center at Dallas, 75390, USA
J Biol Chem 281:34357-64. 2006..These results identify a novel Ca2+-dependent pathway that signals from fibrocystin located in the cell membrane to the nucleus...
- Elucidating the function of primary cilia by conditional gene inactivationThomas Hiesberger
Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, Texas 75390 856, USA
Curr Opin Nephrol Hypertens 14:373-7. 2005..This review discusses recent experimental approaches to determine the function of primary cilia by conditional inactivation of genes crucial for cilia formation...
- Role of the hepatocyte nuclear factor-1beta (HNF-1beta) C-terminal domain in Pkhd1 (ARPKD) gene transcription and renal cystogenesisThomas Hiesberger
Department of Internal Medicine, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, Texas 75390 8856, USA
J Biol Chem 280:10578-86. 2005..Expression of HNF-1alpha in proximal tubules may protect against cystogenesis...
- Mutation of hepatocyte nuclear factor-1beta inhibits Pkhd1 gene expression and produces renal cysts in miceThomas Hiesberger
Division of Nephrology, Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA
J Clin Invest 113:814-25. 2004..HNF-1beta directly regulates the transcription of Pkhd1, and inhibition of PKHD1 gene expression may contribute to the formation of renal cysts in humans with MODY5...
- Regulation of kidney-specific Ksp-cadherin gene promoter by hepatocyte nuclear factor-1betaYun Bai
Division of Nephrology, Department of Internal Medicine, University of Texas Southwestern Medical Center at Dallas, 75390, USA
Am J Physiol Renal Physiol 283:F839-51. 2002..Mutations of HNF-1beta, as occur in humans with inherited renal cysts and diabetes, may cause dysregulated Ksp-cadherin promoter activity...
- HNF-1beta regulates transcription of the PKD modifier gene Kif12Yimei Gong
Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
J Am Soc Nephrol 20:41-7. 2009..Because kinesin-12 family members participate in orienting cell division, downregulation of Kif12 may underlie the abnormal planar cell polarity observed in cystic kidney diseases...
- Kidney-specific inactivation of the KIF3A subunit of kinesin-II inhibits renal ciliogenesis and produces polycystic kidney diseaseFangming Lin
Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
Proc Natl Acad Sci U S A 100:5286-91. 2003..Most generally, the phenotype of Kif3a mutant mice suggests a role for primary cilia in the maintenance of lumen-forming epithelial differentiation...
- A minimal Ksp-cadherin promoter linked to a green fluorescent protein reporter gene exhibits tissue-specific expression in the developing kidney and genitourinary tractXinli Shao
Department of Internal Medicine, The University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390-8856, USA
J Am Soc Nephrol 13:1824-36. 2002..Transgenic mice that express GFP in the mesonephros, metanephros, ureteric bud, and sex ducts may be useful for cell lineage studies...
- Mutations of HNF-1beta inhibit epithelial morphogenesis through dysregulation of SOCS-3Zhendong Ma
Departments of Internal Medicine, Molecular Biology, and Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
Proc Natl Acad Sci U S A 104:20386-91. 2007..Thus, HNF-1beta regulates tubulogenesis by controlling the levels of SOCS-3 expression. Manipulating the levels of SOCS-3 may be a useful therapeutic approach for human diseases induced by HNF-1beta mutations...
- Roles of HNF-1beta in kidney development and congenital cystic diseasesPeter Igarashi
Department of Internal Medicine and Division of Basic Science, University of Texas Southwestern Medical Center, Dallas, 75390, USA
Kidney Int 68:1944-7. 2005....
- Platelet-derived growth factor mediates tyrosine phosphorylation of the cytoplasmic domain of the low Density lipoprotein receptor-related protein in caveolaePhilippe Boucher
Department of Molecular Genetics, University of Texas Southwestern Medical Center, Dallas, Texas 75390 9046, USA
J Biol Chem 277:15507-13. 2002..Taken together, these findings provide a biochemical basis for a cellular signaling pathway that involves apoE and LRP...
- A transcriptional network in polycystic kidney diseaseLionel Gresh
Unité Expression Génétique et Maladies CNRS URA 1644, Departement de Biologie du Developpement, Institut Pasteur, Paris, France
EMBO J 23:1657-68. 2004..This may explain the increased proliferation of cystic cells in MODY5 patients carrying autosomal dominant mutations in HNF1beta...
- Mechanical stimuli induce cleavage and nuclear translocation of the polycystin-1 C terminusVeronique Chauvet
Department of Cellular and Molecular Physiology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
J Clin Invest 114:1433-43. 2004..Polycystin-2, the product of the second gene mutated in ADPKD, modulates the signaling properties of the polycystin-1 CTT. These data reveal a novel pathway by which polycystin-1 transmits messages directly to the nucleus...
- Receptor clustering is involved in Reelin signalingVera Strasser
Max F. Perutz Laboratories, University Departments at the Vienna Biocenter, Department of Medical Biochemistry, University of Vienna, Vienna, Austria
Mol Cell Biol 24:1378-86. 2004....
- The central fragment of Reelin, generated by proteolytic processing in vivo, is critical to its function during cortical plate developmentYves Jossin
Developmental Genetics Unit, University of Louvain Medical School, B1200 Brussels, Belgium
J Neurosci 24:514-21. 2004..They also indicate that events acting in parallel to Dab1 phosphorylation might be required for full activity...