Search Results - (Author, Cooperation:M. Grompe)
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1Latest Papers from Table of Contents or Articles in PressL. Lisowski ; A. P. Dane ; K. Chu ; Y. Zhang ; S. C. Cunningham ; E. M. Wilson ; S. Nygaard ; M. Grompe ; I. E. Alexander ; M. A. Kay
Nature Publishing Group (NPG)
Published 2014Staff ViewPublication Date: 2014-01-07Publisher: Nature Publishing Group (NPG)Print ISSN: 0028-0836Electronic ISSN: 1476-4687Topics: BiologyChemistry and PharmacologyMedicineNatural Sciences in GeneralPhysicsKeywords: Animals ; Capsid/metabolism ; Capsid Proteins/genetics/metabolism ; Carcinoma, Hepatocellular/genetics/pathology ; Cell Line, Tumor ; Cells, Cultured ; Chimera/genetics/metabolism ; Clinical Trials as Topic ; Dependovirus/*genetics/isolation & purification ; Disease Models, Animal ; Female ; Genetic Therapy/*methods ; Genetic Vectors/*genetics ; Hepatocytes/cytology/metabolism/pathology/transplantation ; Heterografts/*metabolism ; Humans ; Liver/cytology/*metabolism/pathology ; Male ; Mice ; Species Specificity ; Transduction, Genetic/*methods ; Transgenes/*geneticsPublished by: -
2Latest Papers from Table of Contents or Articles in PressM. Huch ; C. Dorrell ; S. F. Boj ; J. H. van Es ; V. S. Li ; M. van de Wetering ; T. Sato ; K. Hamer ; N. Sasaki ; M. J. Finegold ; A. Haft ; R. G. Vries ; M. Grompe ; H. Clevers
Nature Publishing Group (NPG)
Published 2013Staff ViewPublication Date: 2013-01-29Publisher: Nature Publishing Group (NPG)Print ISSN: 0028-0836Electronic ISSN: 1476-4687Topics: BiologyChemistry and PharmacologyMedicineNatural Sciences in GeneralPhysicsKeywords: Alleles ; Animals ; Bile Ducts/cytology/metabolism ; Cell Lineage ; Clone Cells/cytology/metabolism ; Culture Media/chemistry/metabolism ; Disease Models, Animal ; Female ; Gene Knock-In Techniques ; Hepatocytes/*cytology/*metabolism/pathology ; Hydrolases/deficiency/genetics ; Liver/cytology/metabolism/pathology ; Liver Diseases/metabolism/pathology ; Male ; Mice ; Multipotent Stem Cells/cytology/metabolism ; Organoids/cytology/transplantation ; Receptors, G-Protein-Coupled/agonists/deficiency/genetics/*metabolism ; *Regeneration ; Stem Cells/*cytology/*metabolism ; Thrombospondins/deficiency/genetics/metabolism ; Tyrosinemias/metabolism/pathology ; *Wnt Signaling PathwayPublished by: -
3Articles: DFG German National LicensesGibson, K. M. ; Schor, D. S. M. ; Gupta, M. ; Guerand, W. S. ; Senephansiri, H. ; Burlingame, T. G. ; Bartels, H. ; Hogema, B. M. ; Bottiglieri, T. ; Froestl, W. ; Snead, O. C. ; Grompe, M. ; Jakobs, C.
Oxford, UK : Blackwell Science, Ltd
Published 2002Staff ViewISSN: 1471-4159Source: Blackwell Publishing Journal Backfiles 1879-2005Topics: MedicineNotes: Metabolite profiling in succinate semialdehyde dehydrogenase (SSADH; Aldh5a1–/–) deficient mice previously revealed elevated γ-hydroxybutyrate (GHB) and total GABA in urine and total brain and liver extracts. In this study, we extend our metabolic characterization of these mutant mice by documenting elevated GHB and total GABA in homogenates of mutant kidney, pancreas and heart. We quantified β-alanine (a GABA homolog and putative neurotransmitter) to address its potential role in pathophysiology. We found normal levels of β-alanine in urine and total homogenates of mutant brain, heart and pancreas, but elevated concentrations in mutant kidney and liver extracts. Amino acid analysis in mutant total brain homogenates revealed no abnormalities except for significantly decreased glutamine, which was normal in mutant liver and kidney extracts. Regional amino acid analysis (frontal cortex, parietal cortex, hippocampus and cerebellum) in mutant mice confirmed glutamine results. Glutamine synthetase protein and mRNA levels in homogenates of mutant mouse brain were normal. We profiled organic acid patterns in mutant brain homogenates to assess brain oxidative metabolism and found normal concentrations of Kreb's cycle intermediates but increased 4,5-dihydroxyhexanoic acid (a postulated derivative of succinic semialdehyde) levels. We conclude that SSADH-deficient mice represent a valid metabolic model of human SSADH deficiency, manifesting focal neurometabolic abnormalities which could provide key insights into pathophysiologic mechanisms.Type of Medium: Electronic ResourceURL: -
4Articles: DFG German National LicensesStaff View
ISSN: 0888-7543Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002Topics: BiologyMedicineType of Medium: Electronic ResourceURL: -
5Articles: DFG German National LicensesStaff View
ISSN: 1432-0584Keywords: Chemical leukemogenesis ; Mice ; VirusSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary The expression of RNA tumor virus was studied in BDF1 mice after leukemogenic treatment with a single dose of methylnitrosourea (MNU) and in leukemic thymuses by a cell ELISA using antibodies against the viral glycoprotein gp 70 and by co-culture for the detection of eco- and xenotropic virus. The majority of the thymomas were positive for gp 70; ecotropic, but not xenotropic infectious virus could be detected in some of them. Early after MNU application the thymus and the bone marrow were positive for gp 70 in some animals. Later, after a phase with positive results with spleen cells, the bone marrow and the spleen were negative again. Only the thymus of some mice were positive during the last weeks before the first leukemias appeared.Type of Medium: Electronic ResourceURL: -
6Articles: DFG German National LicensesStaff View
ISSN: 0168-9525Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002Topics: BiologyType of Medium: Electronic ResourceURL: -
7Articles: DFG German National LicensesStaff View
ISSN: 1432-1203Source: Springer Online Journal Archives 1860-2000Topics: BiologyMedicineNotes: Summary Steroid sulfatase (STS) and arylsulfatase C (ARSC) were studied in fibroblast clones from a 45,X/47,XXX mosaic and from a 69,XXY triploidy with one or two active X chromosomes. The comparison of the 47,XXX with 45,X clones showed an incomplete gene dosage effect (1.8 for STS and 2.0 for ARSC). This was not the case for the triploid clones with different X-inactivation patterns. These results confirm previous reports on the non-inactivation of the STS gene, and establish X linkage and non-inactivation for the ARSC gene as well.Type of Medium: Electronic ResourceURL: -
8Articles: DFG German National LicensesStaff View
ISSN: 1573-2665Source: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Abstract We have studied a knockout mouse with fumarylacetoacetate hydrolase (FAH) deficiency as a model of human hereditary tyrosinaemia type I (HT1). These mice have a phenotype very similar to the human disease, which is characterized by acute hepatic failure, renal tubular disease and hepatocarcinoma. We have previously reported on the efficacy of 2-(2-nitro-4-trifluoromethylbenzyol)-1,3-cyclohexanedione (NTBC) in preventing acute liver disease in HT1 mice. Here we present a progress report on long-term follow up (〉1 year) of high-dose NTBC therapy in combination with tyrosine restriction. In vivo retroviral gene therapy was also effective in abolishing the acute liver failure of HT1. Retrovirally treated mice remained completely healthy and active for 12 months after retroviral gene transfer. However, hepatocarcinoma developed in 2/3 treated animals after 1 year. Southern blot analysis showed that the tumours did not arise from retrovirally transduced hepatocytes but from non-corrected FAH-deficient cells. These results highlight the extreme danger for tumour formation in HT1 and indicate the need for improved gene therapy that leads to the elimination of endogenous FAH-deficient liver cells.Type of Medium: Electronic ResourceURL: -
9Articles: DFG German National LicensesJakobs, P. M. ; Sahaayaruban, P. ; Saito, H. ; Reifsteck, C. ; Olson, S. ; Joenje, H. ; Moses, R. E. ; Grompe, M.
Springer
Published 1996Staff ViewISSN: 1572-9931Source: Springer Online Journal Archives 1860-2000Topics: BiologyMedicineNotes: Abstract Fanconi anemia (FA) is an autosomal recessive disease characterized by birth defects, progressive bone marrow failure and increased risk for leukemia. FA cells display chromosome breakage and increased cell killing in response to DNA crosslinking agents. At least 5 genes have been defined by cell complementation studies, but only one of these, FAC has been cloned to date. Efforts to map and isolate new FA genes by functional complementation have been hapered by the lack of immortalized FA fibroblast cell lines. Here we report the use of a novel immortalization strategy to create 4 new immortalized FA fibroblast lines, including one from the rare complementation group D.Type of Medium: Electronic ResourceURL: