MARKUS C GROMPE

Summary

Affiliation: Oregon Health and Science University
Country: USA

Publications

  1. doi request reprint Tissue stem cells: new tools and functional diversity
    Markus Grompe
    Papé Family Pediatric Research Institute, Oregon Health and Science University, 3181 S W Sam Jackson Park Road, Mail Code L321, Portland, OR 97239 3098, USA
    Cell Stem Cell 10:685-9. 2012
  2. doi request reprint Mice with human livers
    Markus Grompe
    Oregon Stem Cell Center, Department of Pediatrics, Oregon Health and Science University, Portland, Oregon Electronic address
    Gastroenterology 145:1209-14. 2013
  3. ncbi request reprint Adult versus embryonic stem cells: it's still a tie
    Markus Grompe
    Department of Molecular and Medical Genetics L103, 3181 SW Sam Jackson Park Road, Portland, Oregon 97201, USA
    Mol Ther 6:303-5. 2002
  4. ncbi request reprint The Fanconi family adds a fraternal twin
    Markus Grompe
    Oregon Stem Cell Center, Oregon Health Science University, 3181 S W Sam Jackson Park Road, Mail Code L321, Portland, OR 97239, USA
    Dev Cell 12:661-2. 2007
  5. ncbi request reprint The role of bone marrow stem cells in liver regeneration
    Markus Grompe
    Department of Molecular and Medical Genetics L103, Oregon Health and Science University, Portland, Oregon 97239, USA
    Semin Liver Dis 23:363-72. 2003
  6. ncbi request reprint The origin of hepatocytes
    Markus Grompe
    Oregon Stem Cell Center, Oregon Health and Science University, Portland 97239, USA
    Gastroenterology 128:2158-60. 2005
  7. ncbi request reprint Transition of stem cells to therapeutically functional tissue-specific cells
    Markus Grompe
    Oregon Health Science University, 31181 SW Sam Jackson Park Road, Portland, OR 97201, USA
    Ann N Y Acad Sci 961:305-6. 2002
  8. ncbi request reprint Principles of therapeutic liver repopulation
    M Grompe
    Department of Molecular and Medical Genetics, Oregon Health Sciences University, Portland 97201, USA
    Semin Liver Dis 19:7-14. 1999
  9. ncbi request reprint The pathophysiology and treatment of hereditary tyrosinemia type 1
    M Grompe
    Department of Molecular and Medical Genetics, Oregon Health Sciences University, Portland, Oregon 97201, USA
    Semin Liver Dis 21:563-71. 2001
  10. ncbi request reprint Fanconi anemia and DNA repair
    M Grompe
    Department of Molecular and Medical Genetics, Oregon Health Sciences University, 3181 SW Sam Jackson Park Road L103, Portland, OR 97201, USA
    Hum Mol Genet 10:2253-9. 2001

Research Grants

  1. TRANSPLANTATION ASSAY FOR LIVER REPOPULATING CELLS
    Markus Grompe; Fiscal Year: 2005
  2. Pathophysiology of Distal Tyrosine Pathway Disorders
    Markus Grompe; Fiscal Year: 2007
  3. Cell Therapy by In Vivo Fusion
    Markus Grompe; Fiscal Year: 2007
  4. Novel Reagents For Beta Cell Biology
    Markus Grompe; Fiscal Year: 2007
  5. TRANSPLANTATION ASSAY FOR LIVER REPOPULATING CELLS
    Markus Grompe; Fiscal Year: 2009
  6. Treatment of Distal Tyrosine Pathway Disorders
    Markus Grompe; Fiscal Year: 2010
  7. TRANSPLANTATION ASSAY FOR LIVER REPOPULATING CELLS
    Markus Grompe; Fiscal Year: 2010
  8. FASEB Conference: Mechanisms of Liver Growth and Disease
    Markus Grompe; Fiscal Year: 2004
  9. Isolation of Murine Pancreatic Liver Stem Cells
    Markus Grompe; Fiscal Year: 2004
  10. CREATION & ANALYSIS OF A MOUSE MODEL OF TYROSINEMIA
    Markus Grompe; Fiscal Year: 2000

Collaborators

Detail Information

Publications64

  1. doi request reprint Tissue stem cells: new tools and functional diversity
    Markus Grompe
    Papé Family Pediatric Research Institute, Oregon Health and Science University, 3181 S W Sam Jackson Park Road, Mail Code L321, Portland, OR 97239 3098, USA
    Cell Stem Cell 10:685-9. 2012
    ..Furthermore, some tissues have more than one type of stem cell, each used in only particular situations of regenerative stress. Thus, there is no "one size fits all" adult tissue stem cell paradigm...
  2. doi request reprint Mice with human livers
    Markus Grompe
    Oregon Stem Cell Center, Department of Pediatrics, Oregon Health and Science University, Portland, Oregon Electronic address
    Gastroenterology 145:1209-14. 2013
    ..The liver plays a central role in many human-specific biological processes and mice with humanized livers can be used to model human metabolism, liver injury, gene regulation, drug toxicity, and hepatotropic infections. ..
  3. ncbi request reprint Adult versus embryonic stem cells: it's still a tie
    Markus Grompe
    Department of Molecular and Medical Genetics L103, 3181 SW Sam Jackson Park Road, Portland, Oregon 97201, USA
    Mol Ther 6:303-5. 2002
  4. ncbi request reprint The Fanconi family adds a fraternal twin
    Markus Grompe
    Oregon Stem Cell Center, Oregon Health Science University, 3181 S W Sam Jackson Park Road, Mail Code L321, Portland, OR 97239, USA
    Dev Cell 12:661-2. 2007
    ..Like FANCD2, monoubiquitination of FANCI requires the FA core complex. Importantly, FANCI and FANCD2 monoubiquitination is co-dependent, suggesting a novel mechanism in ubiquitin conjugation...
  5. ncbi request reprint The role of bone marrow stem cells in liver regeneration
    Markus Grompe
    Department of Molecular and Medical Genetics L103, Oregon Health and Science University, Portland, Oregon 97239, USA
    Semin Liver Dis 23:363-72. 2003
    ..Second, hematopoietic cells, including lymphocytes, neutrophils, macrophages, and platelets, may provide crucial factors required for efficient healing of damaged liver...
  6. ncbi request reprint The origin of hepatocytes
    Markus Grompe
    Oregon Stem Cell Center, Oregon Health and Science University, Portland 97239, USA
    Gastroenterology 128:2158-60. 2005
  7. ncbi request reprint Transition of stem cells to therapeutically functional tissue-specific cells
    Markus Grompe
    Oregon Health Science University, 31181 SW Sam Jackson Park Road, Portland, OR 97201, USA
    Ann N Y Acad Sci 961:305-6. 2002
  8. ncbi request reprint Principles of therapeutic liver repopulation
    M Grompe
    Department of Molecular and Medical Genetics, Oregon Health Sciences University, Portland 97201, USA
    Semin Liver Dis 19:7-14. 1999
    ..Here we review the current knowledge of this process and discuss the hopeful implications for treatment of liver diseases...
  9. ncbi request reprint The pathophysiology and treatment of hereditary tyrosinemia type 1
    M Grompe
    Department of Molecular and Medical Genetics, Oregon Health Sciences University, Portland, Oregon 97201, USA
    Semin Liver Dis 21:563-71. 2001
    ....
  10. ncbi request reprint Fanconi anemia and DNA repair
    M Grompe
    Department of Molecular and Medical Genetics, Oregon Health Sciences University, 3181 SW Sam Jackson Park Road L103, Portland, OR 97201, USA
    Hum Mol Genet 10:2253-9. 2001
    ..Together, these data suggest that the FA pathway functions primarily as a DNA damage response system, although its exact role (direct involvement in DNA repair versus indirect, facilitating role) has not yet been defined...
  11. ncbi request reprint Liver repopulation for the treatment of metabolic diseases
    M Grompe
    Department of Molecular and Medical Genetics, Department of Pediatrics, Oregon Health Sciences University, Portland 97201, USA
    J Inherit Metab Dis 24:231-44. 2001
    ..Here, the current knowledge regarding therapeutic liver repopulation and the hopeful implications for treatment of liver diseases will be discussed...
  12. ncbi request reprint Therapeutic liver repopulation for the treatment of metabolic liver diseases
    M Grompe
    Dept of Molecular and Medical Genetics, Oregon Health Sciences University, Portland 97201, USA
    Hum Cell 12:171-80. 1999
    ..Here we will review the current knowledge of this process and discuss the hopeful implications for treatment of liver diseases...
  13. ncbi request reprint Principles of therapeutic liver repopulation
    Markus Grompe
    Oregon Stem Cell Center, Oregon Health and Science University, Portland, Oregon, USA
    J Inherit Metab Dis 29:421-5. 2006
    ..In this review, examples of liver repopulation in animals and the implications of these models for clinical cell transplantation will be discussed...
  14. pmc CXCR4 induction in hematopoietic progenitor cells from Fanca(-/-), -c(-/-), and -d2(-/-) mice
    Amy M Skinner
    Department of Pediatrics, Oregon Health and Science University, Portland, OR 97239, USA
    Exp Hematol 36:273-82. 2008
    ..However, inherent repopulation deficits and stem cell attrition during conventional transduction culture prevent therapeutic chimerism...
  15. ncbi request reprint In vivo genetic selection of renal proximal tubules
    Patrice K Held
    Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, OR 97239, USA
    Mol Ther 13:49-58. 2006
    ..These data demonstrate that extensive regeneration of the renal proximal tubule compartment can be achieved through genetic selection of functional cells...
  16. pmc The origin and liver repopulating capacity of murine oval cells
    Xin Wang
    Department of Molecular and Medical Genetics, Oregon Health Sciences University, 3181 Southwest Sam Jackson Park Road, Portland, OR 97239, USA
    Proc Natl Acad Sci U S A 100:11881-8. 2003
    ..We conclude that hepatic oval cells do not originate in bone marrow but in the liver itself, and that they have valuable properties for therapeutic liver repopulation...
  17. ncbi request reprint In vivo correction of murine tyrosinemia type I by DNA-mediated transposition
    Eugenio Montini
    Department of Medical and Molecular Genetics, Oregon Health and Sciences University, Portland, Oregon 97239, USA
    Mol Ther 6:759-69. 2002
    ..Molecular analysis indicated that high-efficiency DNA-mediated transposition into the mouse genome was strictly dependent on the expression of wild-type transposase...
  18. ncbi request reprint Cell fusion is the principal source of bone-marrow-derived hepatocytes
    Xin Wang
    Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, Oregon 97239, USA
    Nature 422:897-901. 2003
    ..We conclude that hepatocytes derived form bone marrow arise from cell fusion and not by differentiation of haematopoietic stem cells...
  19. pmc Sustained phosphorylation of Bid is a marker for resistance to Fas-induced apoptosis during chronic liver diseases
    Arndt Vogel
    Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, Oregon, USA
    Gastroenterology 130:104-19. 2006
    ..To clarify whether stress-induced apoptosis resistance is a general feature of chronic liver diseases, an animal model of chronic cholestasis was examined...
  20. pmc Stem cells and liver regeneration
    Andrew W Duncan
    Oregon Stem Cell Center, Oregon Health and Science University, Portland, Oregon 97239 3098, USA
    Gastroenterology 137:466-81. 2009
    ..This review will consider liver stem cells in the context of each definition...
  21. ncbi request reprint Liver-directed adenoviral gene transfer in murine succinate semialdehyde dehydrogenase deficiency
    Maneesh Gupta
    Molecular and Medical Genetics, Oregon Health and Sciences University, Portland, OR 97239, USA
    Mol Ther 9:527-39. 2004
    ..Our studies provide proof-of-principle that liver-mediated gene therapy has efficacy in treating SSADH deficiency and that hepatic tissue contributes significantly to the pool of GHB within the CNS...
  22. pmc Embryonic lethality after combined inactivation of Fancd2 and Mlh1 in mice
    Henri J van de Vrugt
    Oregon Stem Cell Center, Oregon Health and Science University, Portland, Oregon, USA
    Cancer Res 69:9431-8. 2009
    ....
  23. pmc Robust expansion of human hepatocytes in Fah-/-/Rag2-/-/Il2rg-/- mice
    Hisaya Azuma
    Oregon Stem Cell Center, Oregon Health and Science University, Portland, Oregon 97239, USA
    Nat Biotechnol 25:903-10. 2007
    ..It may also be useful for testing the toxicity of drug metabolites and for evaluating pathogens dependent on human liver cells for replication...
  24. pmc Kinetics of liver repopulation after bone marrow transplantation
    Xin Wang
    Department of Molecular and Medical Genetics, Oregon Health and Sciences University, Portland 97201, USA
    Am J Pathol 161:565-74. 2002
    ..We conclude that hepatocyte replacement by bone marrow cells is a slow and rare event. Significant improvements in the efficiency of this process will be needed before clinical success can be expected...
  25. ncbi request reprint Liver repair by intra- and extrahepatic progenitors
    Craig Dorrell
    Department of Molecular and Medical Genetics, Oregon Stem Cell Center, Oregon Health and Science University, Portland, OR 97239, USA
    Stem Cell Rev 1:61-4. 2005
    ..As is the case for the delivery of intrahepatic progenitors, substantial improvements in the understanding of this process will be needed before clinical application becomes practical...
  26. ncbi request reprint BRCA1 interacts directly with the Fanconi anemia protein FANCA
    Alexandra Folias
    Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, Oregon, USA
    Hum Mol Genet 11:2591-7. 2002
    ..The interaction does not depend on DNA damage, thus FANCA and BRCA1 are constitutively interacting. The demonstrated interaction directly connects BRCA1 to the FA pathway of DNA repair...
  27. pmc ERCC1 is required for FANCD2 focus formation
    Kevin M McCabe
    Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, OR 97239, USA
    Mol Genet Metab 95:66-73. 2008
    ..Our results support a role for ERCC1 after creation of a double strand break for full activation of the Fanconi anemia pathway...
  28. ncbi request reprint Chronic liver disease in murine hereditary tyrosinemia type 1 induces resistance to cell death
    Arndt Vogel
    Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, OR 97239, USA
    Hepatology 39:433-43. 2004
    ..Stress-induced failure of cell death programs may lead to an accumulation of damaged cells and therefore enhance the risk for cancer as observed in HT1 and other chronic liver diseases...
  29. pmc Surface markers for the murine oval cell response
    Craig Dorrell
    Oregon Health and Science University and the Oregon Stem Cell Center, Portland, OR 97239, USA
    Hepatology 48:1282-91. 2008
    ..The subdivision of liver cells using these tools should facilitate the study of the biology of ductal and periductal hepatic cell types, including progenitors...
  30. pmc Ploidy reductions in murine fusion-derived hepatocytes
    Andrew W Duncan
    Oregon Stem Cell Center, Oregon Health and Science University, Portland, Oregon, United States of America
    PLoS Genet 5:e1000385. 2009
    ..The generation of such daughter cells increases liver diversity, which may increase the likelihood of oncogenesis...
  31. ncbi request reprint Signaling networks in hepatic oval cell activation
    Laura Erker
    Oregon Stem Cell Center, Oregon Health Science University, 3181 SW Sam Jackson Park Road, Portland, OR 97239, USA
    Stem Cell Res 1:90-102. 2007
    ..This review describes what is currently known about the factors involved in oval cell activation, proliferation, migration, and differentiation...
  32. doi request reprint Isolation of major pancreatic cell types and long-term culture-initiating cells using novel human surface markers
    Craig Dorrell
    Oregon Health and Science University, Oregon Stem Cell Center, Portland, OR 97239, USA
    Stem Cell Res 1:183-94. 2008
    ..These methods permit the analysis and isolation of functionally distinct pancreatic cell populations with potential for cell therapy...
  33. doi request reprint Tempol protects against oxidative damage and delays epithelial tumor onset in Fanconi anemia mice
    Qing Shuo Zhang
    Oregon Stem Cell Center, Oregon Health and Science University, Portland, OR 97239, USA
    Cancer Res 68:1601-8. 2008
    ..The reduction in oxidative DNA damage in tempol-treated FA fibroblasts and mice suggests that its tumor-delaying function may be attributed to its antioxidant activity...
  34. pmc Adeno-associated virus gene repair corrects a mouse model of hereditary tyrosinemia in vivo
    NICOLE K PAULK
    Oregon Stem Cell Center, Oregon Health and Science University, Portland, OR 97203, USA
    Hepatology 51:1200-8. 2010
    ..Conclusion: AAV-mediated gene repair is feasible in vivo and can functionally correct an appropriate selection-based metabolic liver disease in both adults and neonates...
  35. pmc Non-invasive stem cell therapy in a rat model for retinal degeneration and vascular pathology
    Shaomei Wang
    Casey Eye Institute, Oregon Health and Science University, Portland, Oregon, United States of America
    PLoS ONE 5:e9200. 2010
    ....
  36. pmc Therapeutic liver reconstitution with murine cells isolated long after death
    Laura Erker
    Oregon Stem Cell Center, Oregon Health and Science University, Portland, Oregon 97239, USA
    Gastroenterology 139:1019-29. 2010
    ..For some liver diseases, hepatocyte transplantation could be a viable alternative, but donor cells currently are procured from the same sources as whole organs, and thus the supply is severely limited...
  37. pmc Fancd2-/- mice have hematopoietic defects that can be partially corrected by resveratrol
    Qing Shuo Zhang
    Oregon Stem Cell Center, Department of Pediatrics, Oregon Health and Science University, Portland, OR, USA
    Blood 116:5140-8. 2010
    ..We conclude that Fancd2(-/-) mice have readily quantifiable hematopoietic defects, and that this model is well suited for pharmacologic screening studies...
  38. pmc The ploidy conveyor of mature hepatocytes as a source of genetic variation
    Andrew W Duncan
    Oregon Stem Cell Center, Papé Family Pediatric Research Institute, Oregon Health and Science University, Portland, Oregon 97239, USA
    Nature 467:707-10. 2010
    ..We propose that this mechanism evolved to generate genetic diversity and permits adaptation of hepatocytes to xenobiotic or nutritional injury...
  39. pmc Maleylacetoacetate isomerase (MAAI/GSTZ)-deficient mice reveal a glutathione-dependent nonenzymatic bypass in tyrosine catabolism
    José Manuel Fernández-Cañón
    Department of Molecular and Medical Genetics, Oregon Health Sciences University, Portland, Oregon, USA
    Mol Cell Biol 22:4943-51. 2002
    ..A glutathione-mediated isomerization of MAA to FAA independent of MAAI enzyme was demonstrated in vitro. This nonenzymatic bypass is likely responsible for the lack of a phenotype in nonstressed MAAI mutant mice...
  40. pmc Bone marrow-derived cells fuse with normal and transformed intestinal stem cells
    Adnan Z Rizvi
    Department of Surgery, Division of Hematology and Medical Oncology, and Oregon Cancer Institute, Oregon Stem Cell Center, Oregon Health and Science University, 3181 Southwest Sam Jackson Park Road, Portland, OR 97239, USA
    Proc Natl Acad Sci U S A 103:6321-5. 2006
    ..Fusion of BMDCs with neoplastic epithelium did not result in tumor initiation. Our findings suggest an unexpected role for BMDCs in both regeneration and tumorigenesis of the intestine...
  41. ncbi request reprint Heterozygosity for p53 (Trp53+/-) accelerates epithelial tumor formation in fanconi anemia complementation group D2 (Fancd2) knockout mice
    Scott Houghtaling
    Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, OR 97239, USA
    Cancer Res 65:85-91. 2005
    ..Therefore, Trp53 is required for the S phase checkpoint activation observed in Fancd2 mutant cells. Fancd2(-/-)/Trp53(-/-) cells showed an increase in aneuploidy and had multiple gross chromosomal rearrangements...
  42. ncbi request reprint Interstrand crosslink-induced radials form between non-homologous chromosomes, but are absent in sex chromosomes
    Amy E Hanlon Newell
    Department of Molecular and Medical Genetics, Oregon Health and Science University, MP350, 3181 SW Sam Jackson Park Road, Portland, OR 97239, USA
    DNA Repair (Amst) 3:535-42. 2004
    ..Both X and Y chromosomes were notably not involved in radials. These observations suggest that ICL repair may involve short stretches of homology, resulting in aberrant radial formation in the absence of FA proteins...
  43. ncbi request reprint Myelomonocytic cells are sufficient for therapeutic cell fusion in liver
    Holger Willenbring
    Department of Molecular and Medical Genetics, Oregon Stem Cell Center, Oregon Health and Science University, Portland, Oregon 97239, USA
    Nat Med 10:744-8. 2004
    ..Because stable bone marrow engraftment or HSCs are not required for this process, macrophages or their highly proliferative progenitors provide potential for targeted and well-tolerated cell therapy aimed at organ regeneration...
  44. ncbi request reprint Delineating the hepatocyte's hematopoietic fusion partner
    Holger Willenbring
    Oregon Stem Cell Center and Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, Oregon, USA
    Cell Cycle 3:1489-91. 2004
    ..Now it should be feasible to increase the efficiency and assess the potential of cell fusion for the correction of a broad range of somatic cell types that can be targeted by fusion...
  45. pmc Repair kinetics of genomic interstrand DNA cross-links: evidence for DNA double-strand break-dependent activation of the Fanconi anemia/BRCA pathway
    Andreas Rothfuss
    Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, Oregon 97239, USA
    Mol Cell Biol 24:123-34. 2004
    ..We therefore conclude that the FA/BRCA pathway, while being dispensable for the early events in ICL repair, is activated in S-phase cells after DSB have formed...
  46. ncbi request reprint In vivo correction of murine hereditary tyrosinemia type I by phiC31 integrase-mediated gene delivery
    Patrice K Held
    Molecular and Medical Genetics, Oregon Health and Science University, Portland, OR 97239, USA
    Mol Ther 11:399-408. 2005
    ..The stability of transgene expression, relatively high integration frequency, and significant site specificity that characterize the phiC31 integration system suggest that it may have utility in many gene therapy settings...
  47. ncbi request reprint Pancreatic-hepatic switches in vivo
    Markus Grompe
    Department of Molecular and Medical Genetics L103, Oregon Health and Science University, 3181 SW Sam Jackson Park Road, Portland, OR 97201, USA
    Mech Dev 120:99-106. 2003
    ..These cells may represent a stem cell reservoir with potential in cell therapy applications in the future...
  48. ncbi request reprint Fanconi anemia group A and C double-mutant mice: functional evidence for a multi-protein Fanconi anemia complex
    Meenakshi Noll
    Department of Molecular and Medical Genetics, Oregon Health Sciences University, Portland, OR 97201, USA
    Exp Hematol 30:679-88. 2002
    ..The aim of this study was 1) to determine whether the FA group A and group C genes have identical or partially distinct functions, and 2) to have a better model for human FA...
  49. ncbi request reprint Fancd2 functions in a double strand break repair pathway that is distinct from non-homologous end joining
    Scott Houghtaling
    Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, 97239, USA
    Hum Mol Genet 14:3027-33. 2005
    ..The role of Fancd2 in DSB repair may account for the moderate sensitivity of FA cells to irradiation and FA cells sensitivity to ICLs that are repaired via a DSB intermediate...
  50. ncbi request reprint Bone marrow-derived hepatocytes
    Markus Grompe
    Department of Molecular and Medical Genetics L103, Oregon Health and Science University, 3181 SW Sam Jackson Park Road, Portland, OR 97239, USA
    Novartis Found Symp 265:20-7; discussion 28-34, 92-7. 2005
    ..Fully functional bone marrow-derived hepatocytes indeed exist, but are extremely rare and are generated by cell fusion, not stem cell differentiation...
  51. pmc Epithelial cancer in Fanconi anemia complementation group D2 (Fancd2) knockout mice
    Scott Houghtaling
    Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, Oregon 97239, USA
    Genes Dev 17:2021-35. 2003
    ..The phenotypic overlap between Fancd2-null and Brca2/Fancd1 hypomorphic mice is consistent with a common function for both proteins in the same pathway, regulating genomic stability...
  52. pmc Low therapeutic threshold for hepatocyte replacement in murine phenylketonuria
    Kelly Hamman
    Department of Pediatrics, Oregon Health and Science University, Portland, OR 97239, USA
    Mol Ther 12:337-44. 2005
    ..These data suggest that restoration of phenylalanine homeostasis requires PAH activity in only a minority of hepatocytes...
  53. pmc Slow-onset inhibition of fumarylacetoacetate hydrolase by phosphinate mimics of the tetrahedral intermediate: kinetics, crystal structure and pharmacokinetics
    Raynard L Bateman
    Arthur F Scott Laboratory of Chemistry, Reed College, 3203 SE Woodstock Blvd, Portland, OR 97202, USA
    Biochem J 402:251-60. 2007
    ..These potent inhibitors provide a means to chemically phenocopy the metabolic defects of either HT1 or FAH knockout mice and promise future pharmacological utility for hepatocyte transplantation...
  54. pmc Myeloid lineage progenitors give rise to vascular endothelium
    Alexis S Bailey
    Oregon Stem Cell Center, Center for Hematologic Malignancies, Division of Hematology and Medical Oncology, Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, 97239, USA
    Proc Natl Acad Sci U S A 103:13156-61. 2006
    ..Our findings indicate that endothelial cells are an intrinsic component of myeloid lineage differentiation and underscore the close functional relationship between the hematopoietic and vascular systems...
  55. ncbi request reprint Therapeutic intervention in mice deficient for succinate semialdehyde dehydrogenase (gamma-hydroxybutyric aciduria)
    Maneesh Gupta
    Department of Molecular and Medical Genetics and Pediatrics, Oregon Health and Science University, 2525 SW 3rd Avenue, MP 350, Portland, OR 97201, USA
    J Pharmacol Exp Ther 302:180-7. 2002
    ..We conclude that taurine and NCS-382 may have therapeutic relevance in human SSADH deficiency and that the poor clinical efficacy of VGB in this disease may relate to an inability to decrease brain GHB concentrations...
  56. ncbi request reprint Mutational spectrum of the succinate semialdehyde dehydrogenase (ALDH5A1) gene and functional analysis of 27 novel disease-causing mutations in patients with SSADH deficiency
    Shinjiro Akaboshi
    Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland 97201, USA
    Hum Mutat 22:442-50. 2003
    ....
  57. ncbi request reprint AAV serotype 2 vectors preferentially integrate into active genes in mice
    Hiroyuki Nakai
    Department of Pediatrics, Stanford University School of Medicine, 300 Pasteur Dr Rm G305A, Stanford, California 94305, USA
    Nat Genet 34:297-302. 2003
    ....
  58. ncbi request reprint Helper-independent and AAV-ITR-independent chromosomal integration of double-stranded linear DNA vectors in mice
    Hiroyuki Nakai
    Department of Pediatrics, Stanford University School of Medicine, Stanford, California 94305, USA
    Mol Ther 7:101-11. 2003
    ..In addition, they may provide a clue for developing new nonviral integrating gene delivery vector systems...
  59. pmc CDX2 in the formation of the trophectoderm lineage in primate embryos
    Hathaitip Sritanaudomchai
    Oregon National Primate Research Center, Oregon Health and Science University, Beaverton, OR, USA
    Dev Biol 335:179-87. 2009
    ..These results provide evidence that CDX2 plays an essential role in functional TE formation during primate embryonic development...
  60. ncbi request reprint Embryonic versus adult stem cell pluripotency: in liver only fusion matters
    Holger Willenbring
    Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, Oregon 97239, USA
    J Assist Reprod Genet 20:393-4. 2003
  61. pmc Large-scale molecular characterization of adeno-associated virus vector integration in mouse liver
    Hiroyuki Nakai
    Department of Pediatrics, 300 Pasteur Dr, Grant Bldg, Rm S374, Stanford University School of Medicine, Stanford, CA 94305, USA
    J Virol 79:3606-14. 2005
    ..Thus, the present study provides new insights into the risk of rAAV-mediated insertional mutagenesis and the mechanisms of rAAV integration...
  62. ncbi request reprint Complete hepatic regeneration after somatic deletion of an albumin-plasminogen activator transgene. 1991
    Markus Grompe
    Department of Molecular and Medical Genetics, L103, 3181 SW Sam Jackson Park Road, Portland, OR 97201, USA
    J Hepatol 37:422-4. 2002
  63. pmc Natural gene therapy in monozygotic twins with Fanconi anemia
    Anuj Mankad
    Department of Molecular and Medical Genetics, Oregon Health and Science University, 3181 SW Sam Jackson Park Rd, Portland, OR 97239, USA
    Blood 107:3084-90. 2006
    ..This finding suggests that treating FA patients with gene therapy might require transduction of only a few hematopoietic stem cells...
  64. doi request reprint Validation of Fanconi anemia complementation Group A assignment using molecular analysis
    Nabil N Moghrabi
    Department of Molecular and Medical Genetics, Clinical Genetics Laboratories, Oregon Health and Science University, 3181 S W Sam Jackson Park Road, Portland, OR 97239, USA
    Genet Med 11:183-92. 2009
    ....

Research Grants39

  1. TRANSPLANTATION ASSAY FOR LIVER REPOPULATING CELLS
    Markus Grompe; Fiscal Year: 2005
    ..In addition, we will explore the properties of liver repopulation by transplanted hematopoietic stem cells (HSCs). ..
  2. Pathophysiology of Distal Tyrosine Pathway Disorders
    Markus Grompe; Fiscal Year: 2007
    ..In aim 2, we will use small molecule inhibitors of Fah to select for genetically modified hepatocytes in vivo. In aim 3, the pathophysiology of hepatic cancer in HT 1 and strategies for its chemoprevention will be studied. ..
  3. Cell Therapy by In Vivo Fusion
    Markus Grompe; Fiscal Year: 2007
    ..Aim 3 is geared toward enhancing the efficiency of in vivo fusion and thereby increasing the number of bone marrow derived hepatocytes. Fusogenic viral envelope proteins will be used to artificially induce cell fusion. ..
  4. Novel Reagents For Beta Cell Biology
    Markus Grompe; Fiscal Year: 2007
    ..Definition of both families of targets is required for a complete understanding of beta cell biology and pathophysiology ..
  5. TRANSPLANTATION ASSAY FOR LIVER REPOPULATING CELLS
    Markus Grompe; Fiscal Year: 2009
    ..In addition, the creation of a widely usable murine model for repopulation with human hepatocytes will facilitate translational studies in all areas of liver biology. ..
  6. Treatment of Distal Tyrosine Pathway Disorders
    Markus Grompe; Fiscal Year: 2010
    ..In addition, a broadly applicable platform for pharmacological growth selection of genetically modified hepatocytes is being developed. ..
  7. TRANSPLANTATION ASSAY FOR LIVER REPOPULATING CELLS
    Markus Grompe; Fiscal Year: 2010
    ..In addition, the creation of a widely usable murine model for repopulation with human hepatocytes will facilitate translational studies in all areas of liver biology. ..
  8. FASEB Conference: Mechanisms of Liver Growth and Disease
    Markus Grompe; Fiscal Year: 2004
    ..abstract_text> ..
  9. Isolation of Murine Pancreatic Liver Stem Cells
    Markus Grompe; Fiscal Year: 2004
    ..Monoclonal antibodies useful for FACS sorting of pancreatic cells will be generated. We will apply cell-sorting methods to enrich pancreatic liver stem cells. ..
  10. CREATION & ANALYSIS OF A MOUSE MODEL OF TYROSINEMIA
    Markus Grompe; Fiscal Year: 2000
    ..Accumulation of the hepatotoxic substrates of FAH will be induced by administration of substrate precursor or specific FAH inhibitors and in vivo selection will be monitored. ..
  11. TRANSPLANTATION ASSAY FOR LIVER REPOPULATING CELLS
    Markus Grompe; Fiscal Year: 2000
    ..By crossbreeding to SCID mice, we will also create an immune deficient FAH mutant mouse. These animals will be used to develop an analogous liver repopulation assay for xenogeneic donor hepatocytes from rat, primates and humans. ..
  12. PATHOPHYSIOLOGY AND TREATMENT OF DISORDERS IN THE DISTAL
    Markus Grompe; Fiscal Year: 2004
    ....
  13. TRANSPLANTATION ASSAY FOR LIVER REPOPULATING CELLS
    Markus Grompe; Fiscal Year: 2009
    ..In addition, the creation of a widely usable murine model for repopulation with human hepatocytes will facilitate translational studies in all areas of liver biology. ..