title: Organ specific optical imaging of mitochondrial redox state in a rodent model of hereditary hemorrhagic telangiectasia-1.
abstract: Hereditary Hemorrhagic Telangiectasia-1 (HHT-1) is a vascular disease caused by mutations in the endoglin (Eng)/CD105 gene. The objective of this study was to quantify the oxidative state of a rodent model of HHT-1 using an optical imaging technique. We used a cryofluorescence imaging instrument to quantitatively assess tissue metabolism in this model. Mitochondrial redox ratio (FAD/NADH), FAD RR, was used as a quantitative marker of the metabolic status and was examined in the kidneys, and eyes of wild-type and Eng +/- mice. Kidneys and eyes from wild-type P21, 6W, and 10M old mice showed, respectively, a 9% (±2), 24% (±0.4), 15% (±1), and 23% (±4), 33% (±0.6), and 30% (±2) change in the mean FAD RR compared to Eng +/- mice at the same age. Thus, endoglin haploinsufficiency is associated with less oxidative stress in various organs and mitigation of angiogenesis.
authors.affiliation: Department of Electrical Engineering, University of Wisconsin Milwaukee, Milwaukee, WI, USA.
authors.forename: Zahra
authors.initials: Z
authors.lastname: Ghanian
authors.forename: Sepideh
authors.initials: S
authors.lastname: Maleki
authors.forename: SunYoung
authors.initials: S
authors.lastname: Park
authors.forename: Christine M
authors.initials: CM
authors.lastname: Sorenson
authors.forename: Nader
authors.initials: N
authors.lastname: Sheibani
authors.forename: Mahsa
authors.initials: M
authors.lastname: Ranji
mesh_terms: D000818:Animals; D002478:Cells, Cultured; D004195:Disease Models, Animal; D000071063:Endoglin; D005123:Eye / Q000378:metabolism / Q000473:pathology; D005182:Flavin-Adenine Dinucleotide / Q000378:metabolism*; D005615:Freezing; D021621:Imaging, Three-Dimensional; D047908:Intracellular Signaling Peptides and Proteins / Q000172:deficiency / Q000235:genetics; D007668:Kidney / Q000378:metabolism / Q000473:pathology; D008168:Lung / Q000378:metabolism / Q000473:pathology; D008822:Mice, Transgenic; D008928:Mitochondria / Q000378:metabolism; D009243:NAD / Q000378:metabolism*; D061848:Optical Imaging / Q000379:methods*; D018384:Oxidative Stress; D017382:Reactive Oxygen Species / Q000378:metabolism*; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000378:metabolism* / Q000473:pathology*
chemical_list: D000071063:Endoglin; C502004:Eng protein, mouse; D047908:Intracellular Signaling Peptides and Proteins; D017382:Reactive Oxygen Species; D009243:NAD; D005182:Flavin-Adenine Dinucleotide
keywords: Endoglin; FAD; Hereditary Hemorrhagic Telangiectasia-1; NADH; optical imaging; oxidative stress; oxygen-induced ischemic retinopathy; redox ratio