197 results on '"Airik, Rannar"'
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2. Persistent DNA damage underlies tubular cell polyploidization and progression to chronic kidney disease in kidneys deficient in the DNA repair protein FAN1
3. Primary coenzyme Q10 nephropathy, a potentially treatable form of steroid-resistant nephrotic syndrome
4. Endothelial cells drive organ fibrosis in mice by inducing expression of the transcription factor SOX9
5. Repression of Sox9 by Jag1 Is Continuously Required to Suppress the Default Chondrogenic Fate of Vascular Smooth Muscle Cells
6. ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption
7. Abstract GS.06: The Fibrogenic Transcription Factor Sox9 Controls Mesenchymal Activation Of Endothelial Cells And Drives Fibrotic Disease
8. Mitochondrial ROS Triggers KIN Pathogenesis in FAN1-Deficient Kidneys
9. Treatment with 2,4-Dihydroxybenzoic Acid Prevents FSGS Progression and Renal Fibrosis in Podocyte-Specific Coq6 Knockout Mice
10. Premature Aging and Reduced Cancer Incidence Associated with Near-Complete Body-WideMycInactivation
11. Premature Aging and Reduced Cancer Incidence Associated With Body-Wide Loss of Myc
12. Disruption of Multiple Overlapping Functions Following Stepwise Inactivation of the Extended Myc Network
13. Osteoclast stimulation factor 1 (Ostf1) KNOCKOUT increases trabecular bone mass in mice
14. Mitigation of portal fibrosis and cholestatic liver disease in ANKS6 ‐deficient livers by macrophage depletion
15. BiallelicANKS6mutations cause late-onset ciliopathy with chronic kidney disease through YAP dysregulation
16. Mutation analysis in Bardet–Biedl syndrome by DNA pooling and massively parallel resequencing in 105 individuals
17. COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness
18. Loss of Sox9 in the periotic mesenchyme affects mesenchymal expansion and differentiation, and epithelial morphogenesis during cochlea development in the mouse
19. Biallelic ANKS6 mutations cause late-onset ciliopathy with chronic kidney disease through YAP dysregulation.
20. Mutation analysis of 18 nephronophthisis associated ciliopathy disease genes using a DNA pooling and next generation sequencing strategy
21. Loss ofAnks6leads to YAP deficiency and liver abnormalities
22. Hydroureternephrosis due to loss of Sox9-regulated smooth muscle cell differentiation of the ureteric mesenchyme
23. Tbx18 regulates the development of the ureteral mesenchyme
24. Gene expression analysis of Gata3−/− mice by using cDNA microarray technology
25. Roscovitine blocks collecting duct cyst growth in Cep164-deficient kidneys
26. Delayed onset of smooth muscle cell differentiation leads to hydroureter formation in mice with conditional loss of the zinc finger transcription factor geneGata2in the ureteric mesenchyme
27. Loss of Anks6 leads to YAP deficiency and liver abnormalities.
28. The prepattern transcription factor Irx3 directs nephron segment identity
29. Delayed onset of smooth muscle cell differentiation leads to hydroureter formation in mice with conditional loss of the zinc finger transcription factor gene Gata2 in the ureteric mesenchyme.
30. Whole exome sequencing identifies causative mutations in the majority of consanguineous or familial cases with childhood-onset increased renal echogenicity
31. SDCCAG8 Interacts with RAB Effector Proteins RABEP2 and ERC1 and Is Required for Hedgehog Signaling
32. FAT1 mutations cause a glomerulotubular nephropathy
33. SDCCAG8 Interacts with RAB Effector Proteins RABEP2 and ERC1 and Is Required for Hedgehog Signaling
34. A FANCD2/FANCI-Associated Nuclease 1-Knockout Model Develops Karyomegalic Interstitial Nephritis
35. FAT1 mutations cause a glomerulotubular nephropathy
36. Whole exome sequencing identifies causative mutations in the majority of consanguineous or familial cases with childhood-onset increased renal echogenicity
37. DCDC2 mutations cause a renal-hepatic ciliopathy by disrupting Wnt signaling
38. DCDC2 mutations cause a renal-hepatic ciliopathy by disrupting Wnt signaling
39. Defects in the IFT-B Component IFT172 Cause Jeune and Mainzer-Saldino Syndromes in Humans
40. Zebrafish Ciliopathy Screen Plus Human Mutational Analysis Identifies C21orf59 and CCDC65 Defects as Causing Primary Ciliary Dyskinesia
41. Mitochondrial Reactive Oxygen Species (ROS) Triggers Karyomegalic Interstitial Nephritis (KIN) Pathogenesis in FAN1-Deficient Kidneys
42. DCDC2 Mutations Cause a Renal-Hepatic Ciliopathy by Disrupting Wnt Signaling
43. Renal-Retinal Ciliopathy Gene Sdccag8 Regulates DNA Damage Response Signaling
44. Whole-exome resequencing distinguishes cystic kidney diseases from phenocopies in renal ciliopathies
45. Upk3b Is Dispensable for Development and Integrity of Urothelium and Mesothelium
46. Renal-Retinal Ciliopathy Gene Sdccag8 Regulates DNA Damage Response Signaling
47. Nephric duct insertion requires EphA4/EphA7 signaling from the pericloacal mesenchyme
48. SDCCAG8 Regulates Pericentriolar Material Recruitment and Neuronal Migration in the Developing Cortex
49. Whole-exome resequencing distinguishes cystic kidney diseases from phenocopies in renal ciliopathies
50. Effect of vanillic acid on COQ6 mutants identified in patients with coenzyme Q10 deficiency
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