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Autor
Adori, Csaba 1 Alten, Florian 1 Andersson, Emma R 1 Baumgärtel, Karsten 1 Bryja, Vitezslav 1 Dawit, Feven 1 Fahnehjelm, Kristina Teär 1 Fischler, Björn 1 Fredriksson, Linda 1 Hankeova, Simona 1 Hassan, Mohamed G 1 Hensens, Naomi 1 Jheon, Andrew 1 Johansson, Bengt R 1 Kaiser, Jozef 1 Kavkova, Michaela 1 Laznovsky, Jakub 1 Lendahl, Urban 1 Mangold, Katrin 1 Salplachta, Jakub 1
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Pracoviště
CEITEC Central European Institute of ... 1 Department of Cell and Molecular Biol... 1 Department of Clinical Neuroscience K... 1 Department of Experimental Biology Ma... 1 Department of Medical Biochemistry an... 1 Department of Neuroscience Karolinska... 1 Department of Ophthalmology Universit... 1 Department of Orthodontics Faculty of... 1 Department of Pediatric Ophthalmology... 1 Department of Pediatrics Clinical Sci... 1 EM Unit Institute of Biomedicine Univ... 1 Travere Therapeutics San Diego CA USA 1 University of Applied Sciences Utrech... 1 University of San Francisco San Franc... 1
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Autor
Adori, Csaba 1 Alten, Florian 1 Andersson, Emma R 1 Baumgärtel, Karsten 1 Bryja, Vitezslav 1 Dawit, Feven 1 Fahnehjelm, Kristina Teär 1 Fischler, Björn 1 Fredriksson, Linda 1 Hankeova, Simona 1 Hassan, Mohamed G 1 Hensens, Naomi 1 Jheon, Andrew 1 Johansson, Bengt R 1 Kaiser, Jozef 1 Kavkova, Michaela 1 Laznovsky, Jakub 1 Lendahl, Urban 1 Mangold, Katrin 1 Salplachta, Jakub 1
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Pracoviště
CEITEC Central European Institute of ... 1 Department of Cell and Molecular Biol... 1 Department of Clinical Neuroscience K... 1 Department of Experimental Biology Ma... 1 Department of Medical Biochemistry an... 1 Department of Neuroscience Karolinska... 1 Department of Ophthalmology Universit... 1 Department of Orthodontics Faculty of... 1 Department of Pediatric Ophthalmology... 1 Department of Pediatrics Clinical Sci... 1 EM Unit Institute of Biomedicine Univ... 1 Travere Therapeutics San Diego CA USA 1 University of Applied Sciences Utrech... 1 University of San Francisco San Franc... 1
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NLK
Directory of Open Access Journals
od 2009
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od 2009
PubMed Central
od 2009
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od 2009
ProQuest Central
od 2009-04-01
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od 2009-01-01
Open Access Digital Library
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Medline Complete (EBSCOhost)
od 2011-01-01
Health & Medicine (ProQuest)
od 2009-04-01
ROAD: Directory of Open Access Scholarly Resources
od 2008
PubMed
36345711
DOI
10.15252/emmm.202215809
Knihovny.cz E-zdroje
Spontaneous bleeds are a leading cause of death in the pediatric JAG1-related liver disease Alagille syndrome (ALGS). We asked whether there are sex differences in bleeding events in patients, whether Jag1Ndr/Ndr mice display bleeds or vascular defects, and whether discovered vascular pathology can be confirmed in patients non-invasively. We performed a systematic review of patients with ALGS and vascular events following PRISMA guidelines, in the context of patient sex, and found significantly more girls than boys reported with spontaneous intracranial hemorrhage. We investigated vascular development, homeostasis, and bleeding in Jag1Ndr/Ndr mice, using retina as a model. Jag1Ndr/Ndr mice displayed sporadic brain bleeds, a thin skull, tortuous blood vessels, sparse arterial smooth muscle cell coverage in multiple organs, which could be aggravated by hypertension, and sex-specific venous defects. Importantly, we demonstrated that retinographs from patients display similar characteristics with significantly increased vascular tortuosity. In conclusion, there are clinically important sex differences in vascular disease in ALGS, and retinography allows non-invasive vascular analysis in patients. Finally, Jag1Ndr/Ndr mice represent a new model for vascular compromise in ALGS.
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