Research Topics
Species | John BisslerSummaryAffiliation: Cincinnati Children's Hospital Medical Center Country: USA Publications
Research Grants
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Detail Information
Publications
Human TSC-associated renal angiomyolipoma cells are hypersensitive to ER stressBrian J Siroky
Cincinnati Children s Hospital Medical Center, Division of Nephrology and Hypertension, 3333 Burnet Ave, MLC 7022, Cincinnati, OH 45229 3039
Am J Physiol Renal Physiol 303:F831-44. 2012..These results suggest that human angiomyolipoma cells are uniquely susceptible to agents that exacerbate ER stress and that additional synergy may be achievable with targeted combination therapy...- Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosisJohn J Bissler
Division of Nephrology and Hypertension, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
N Engl J Med 358:140-51. 2008..The drug sirolimus suppresses mTOR signaling... - A mechanistic approach to inherited polycystic kidney diseaseJohn J Bissler
Division of Nephrology and Hypertension, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
Pediatr Nephrol 20:558-66. 2005..Renal cystogenesis may be the result of a disruption of a critical feedback loop that regulates tissue morphology based on the epithelial cell environment... - Renal angiomyolipomataJohn J Bissler
Division of Nephrology and Hypertension, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
Kidney Int 66:924-34. 2004..The purpose of this article is to update the nephrologist on the molecular understanding of renal angiomyolipomata and for the possibilities of pharmacologic therapy in the future... - Triplex DNA and human diseaseJohn J Bissler
Department of Pediatrics, Division of Nephrology, Cincinnati Children s Hospital Medical Center and the University of Cincinnati College of Medicine, Cincinnati, OH 45229 3039, USA
Front Biosci 12:4536-46. 2007..Some alternative DNA structures are associated with human diseases, and this review focuses on disease-associated polypurine polypyrimidine mirror repeat sequences... - Glomerulocystic kidney diseaseJohn J Bissler
Division of Nephrology and Hypertension, Cincinnati Children s Hospital Medical Center, MLC 7022, 3333 Burnet Avenue, Cincinnati, OH 45229 3039, USA
Pediatr Nephrol 25:2049-56; quiz 2056-9. 2010.... - Characterization of Fabry disease in 352 pediatric patients in the Fabry RegistryRobert J Hopkin
Division of Human Genetics, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
Pediatr Res 64:550-5. 2008..Thus, many pediatric Fabry patients report early symptoms, particularly pain, gastrointestinal symptoms, and impaired quality of life. Some children experience major complications during the pediatric years... - Stathmin-deficient mice develop fibrosis and show delayed recovery from ischemic-reperfusion injuryKamyar Zahedi
Division of Nephrology and Hypertension, Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
Am J Physiol Renal Physiol 290:F1559-67. 2006.... - Reduction of postembolization syndrome after ablation of renal angiomyolipomaJohn J Bissler
Division of Nephrology and Department of Radiology, Children s Hospital Medical Center, Cincinnati, OH, USA
Am J Kidney Dis 39:966-71. 2002..The use of a short-term tapering dose of prednisone appeared to reduce PES compared with the reported literature and improved patient comfort... - Tuberous sclerosis complex: renal imaging findingsKeith A Casper
Department of Radiology, Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229-3039, USA
Radiology 225:451-6. 2002..13). CONCLUSION: Both angiomyolipomas and cysts occur commonly in pediatric patients with TSC and tend to increase in size and number with increasing age. Angiomyolipomas are more common than cysts and tend to be numerous... - Embolization of renal angiomyolipomata in patients with tuberous sclerosis complexJarrod M Williams
Division of Radiology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Am J Kidney Dis 47:95-102. 2006..CONCLUSION: Selective embolization decreases tumor size, prevents hemorrhage, and preserves kidney function in patients with tuberous sclerosis with renal angiomyolipomata... - Automated algorithm for quantifying the extent of cystic change on volumetric chest CT: initial results in LymphangioleiomyomatosisVincent J Schmithorst
Department of Radiology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH, USA
AJR Am J Roentgenol 192:1037-44. 2009..The purpose of our study was to develop a new method for quantifying the severity of cystic lung disease using chest CT and to evaluate this method in patients with lymphangioleiomyomatosis (LAM)... - Renal involvement in tuberous sclerosis complex and von Hippel-Lindau disease: shared disease mechanisms?Brian J Siroky
Division of Nephrology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH, USA
Nat Clin Pract Nephrol 5:143-56. 2009..This Review summarizes the pathophysiologic and therapeutic overlap between tuberous sclerosis complex and von Hippel-Lindau disease and highlights the results of recent drug trials in these settings... - Comparative evaluation of alpha-galactosidase A infusions for treatment of Fabry diseaseRobert J Hopkin
Division and Program in Human Genetics, Cincinnati Children's Research Foundation, Cincinnati, Ohio 45229-3039, USA
Genet Med 5:144-53. 2003..In addition, the need for additional long-term data is emphasized because this is not attainable in short-term trials for a chronic disease... - RecQ and RecG helicases have distinct roles in maintaining the stability of polypurine.polypyrimidine sequencesBradley P Dixon
Division of Nephrology and Hypertension, Cincinnati Children s Hospital Medical Center, MLC 7022, 3333 Burnet Avenue, Cincinnati, OH 45229 3039, United States
Mutat Res 643:20-8. 2008..5 kb Pu.Py tract containing multiple mirror repeats. These results support a two-tiered model where RecQ facilitates fork progression through triplex-forming tracts and, failing processivity, RecG is critical for replication fork restart... - PKD1 intron 21: triplex DNA formation and effect on replicationHiren P Patel
Division of Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229-3039, USA
Nucleic Acids Res 32:1460-8. 2004..During DNA replication, the G-rich template of the PKD1 Pu.Py tracts may form a triplex structure with the nascent strand, thereby blocking replication and potentially leading to recombination and mutation... - Spermidine/spermine N1-acetyltransferase overexpression in kidney epithelial cells disrupts polyamine homeostasis, leads to DNA damage, and causes G2 arrestKamyar Zahedi
Division of Nephrology and Hypertension, Children s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH 45267, USA
Am J Physiol Cell Physiol 292:C1204-15. 2007..We propose that in kidneys subjected to IRI, one mechanism through which increased expression of SSAT may cause cellular injury and organ damage is through induction of DNA damage and the disruption of cell cycle... - DNA lesion-specific co-localization of the Mre11/Rad50/Nbs1 (MRN) complex and replication protein A (RPA) to repair fociJacob G Robison
Department of Environmental Health, University of Cincinnati College of Medicine, Cincinnati, Ohio 45267, USA
J Biol Chem 280:12927-34. 2005.... - Frequency and imaging appearance of hepatic angiomyolipomas in pediatric and adult patients with tuberous sclerosisBradley L Fricke
Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229-3039, USA
AJR Am J Roentgenol 182:1027-30. 2004..CONCLUSION: Multiple hepatic angiomyolipomas are often found in patients with tuberous sclerosis, particularly in those with bilateral diffuse renal angiomyolipomas... - Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseasesLisa R Young
Division of Pulmonary Medicine, Cincinnati Children s Hospital Medical Center, Cincinnati, OH, USA
Chest 138:674-81. 2010..The purpose of this study was to determine the prospective diagnostic usefulness of a serologic test for vascular endothelial growth factor-D (VEGF-D), a lymphangiogenic growth factor... - Increased cancer risk of augmentation cystoplasty: possible role for hyperosmolal microenvironment on DNA damage recognitionBradley P Dixon
Division of Nephrology and Hypertension, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
Mutat Res 670:88-95. 2009..Failure of such protective adaptations in the tissues used for augmentation cystoplasties may place these tissues at increased risk for malignancy... - Renal, hepatic, and marrow dysfunction in a patient with chronic renal insufficiencyRebecca C Brady
Department of Pediatrics, Division of Nephrology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
Pediatr Nephrol 18:293-6. 2003..Doxycycline therapy and aggressive supportive care led to complete recovery... - Cell cycle-and proteasome-dependent formation of etoposide-induced replication protein A (RPA) or Mre11/Rad50/Nbs1 (MRN) complex repair fociJacob G Robison
Division of Nephrology and Hypertension, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
Cell Cycle 6:2399-407. 2007..These data suggest that in response to DNA DSBs, cell cycle-preferred repair pathways differentially engage RPA and the MRN complex in repair foci... - Replication protein A is required for etoposide-induced assembly of MRE11/RAD50/NBS1 complex repair fociJacob G Robison
Department of Environmental Health, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
Cell Cycle 6:2408-16. 2007..These data support a role for RPA as an initial signal/sensor for DNA damage that facilitates recruitment of MRN and ATM/ATR to sites of damage, where they then work together to fully activate the DNA damage response... - Unstable spinocerebellar ataxia type 10 (ATTCT*(AGAAT) repeats are associated with aberrant replication at the ATX10 locus and replication origin-dependent expansion at an ectopic site in human cellsGuoqi Liu
Department of Biochemistry and Molecular Biology, Boonshoft School of Medicine, Wright State University, Dayton, Ohio 45435, USA
Mol Cell Biol 27:7828-38. 2007..These results implicate replication origin activity as one molecular mechanism associated with the instability of (ATTCT)n tracts that are longer than normal length... - Unpaired structures in SCA10 (ATTCT)n.(AGAAT)n repeatsVladimir N Potaman
Laboratory of DNA Structure and Function, Center for Genome Research, Institute of Biosciences and Technology, Texas A and M University System Health Sciences Center, Houston, TX 77030-3303, USA
J Mol Biol 326:1095-111. 2003..A model is proposed in which unscheduled or aberrant DNA replication is a critical step in the expansion mutation... - Apolipoprotein J/clusterin prevents a progressive glomerulopathy of agingMark E Rosenberg
Division of Renal Diseases and Hypertension, Department of Medicine, University of Minnesota, UMHC 736, 516 Delaware Street SE, Minneapolis, MN 55455, USA
Mol Cell Biol 22:1893-902. 2002..These results establish a protective role of apoJ/clusterin against chronic glomerular kidney disease and support the hypothesis that apoJ/clusterin modifies immune complex metabolism and disposal...
Research Grants
- Utility of Rapamycin for the Treatment of Renal Angiomy*John Bissler; Fiscal Year: 2004..Successful completion of the aim of this study will help to establish tuberous sclerosis as a valuable model for targeted molecular therapy for neoplasia. ..
- DNA Replication Fork: Pausing, Recombination and DiseaseJohn Bissler; Fiscal Year: 2007..By understanding the effects of these sequences on the fidelity of DNA replication, therapeutic inroads into delaying disease onset can be made. ..