Increased plasma membrane cholesterol in cystic fibrosis cells correlates with CFTR genotype and depends on de novo cholesterol synthesis

Autor: Sonawane Nitin D, Previs Stephen F, Jiang Dechen, Ruddy Jennifer, Manson Mary E, West Richard H, Fang Danjun, Burgess James D, Kelley Thomas J

Publikováno v: Respiratory Research, Vol 11, Iss 1, p 61 (2010)

Abstract Background Previous observations demonstrate that Cftr-null cells and tissues exhibit alterations in cholesterol processing including perinuclear cholesterol accumulation, increased de novo synthesis, and an increase in plasma membrane chole

Externí odkaz: https://doaj.org/article/addca608fea448e6b5c66fc5036a8b06

Ultrasound-mediated gene delivery specifically targets liver sinusoidal endothelial cells for sustained FVIII expression in hemophilia A mice.

Autor: Lawton SM; Seattle Children's Research Institute, Seattle, WA, USA., Manson MA; Seattle Children's Research Institute, Seattle, WA, USA., Fan MN; Seattle Children's Research Institute, Seattle, WA, USA., Chao TY; Seattle Children's Research Institute, Seattle, WA, USA., Chen CY; Seattle Children's Research Institute, Seattle, WA, USA., Kim P; Seattle Children's Research Institute, Seattle, WA, USA., Campbell C; Seattle Children's Research Institute, Seattle, WA, USA., Cai X; Seattle Children's Research Institute, Seattle, WA, USA., Vander Kooi A; Seattle Children's Research Institute, Seattle, WA, USA., Miao CH; Seattle Children's Research Institute, Seattle, WA, USA; University of Washington, Seattle, WA, USA. Electronic address: carol.miao@seattlechildrens.org.

Publikováno v: Molecular therapy : the journal of the American Society of Gene Therapy [Mol Ther] 2024 Apr 03; Vol. 32 (4), pp. 969-981. Date of Electronic Publication: 2024 Feb 09.

Extracellular matrix dysfunction in Sorsby patient-derived retinal pigment epithelium.

Autor: Engel AL; Department of Ophthalmology, University of Washington, Seattle, WA, 98109, USA., Wang Y; Department of Ophthalmology, West Virginia University, Morgantown, WV, 26506, USA; Department of Biochemistry, West Virginia University, Morgantown, WV, 26506, USA., Khuu TH; Department of Ophthalmology, University of Washington, Seattle, WA, 98109, USA., Worrall E; Department of Ophthalmology, University of Washington, Seattle, WA, 98109, USA., Manson MA; Department of Ophthalmology, University of Washington, Seattle, WA, 98109, USA., Lim RR; Department of Ophthalmology, University of Washington, Seattle, WA, 98109, USA., Knight K; Department of Ophthalmology, University of Washington, Seattle, WA, 98109, USA., Yanagida A; Department of Ophthalmology, University of Washington, Seattle, WA, 98109, USA., Qi JH; Department of Ophthalmic Research, Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH, 44106, USA., Ramakrishnan A; Center for Blood Cancers and Oncology, St. David's South Austin Medical Center, Austin, TX, 78704, USA., Weleber RG; Casey Eye Institute, Oregon Health & Science University, Portland, OR, 97201, USA., Klein ML; Casey Eye Institute, Oregon Health & Science University, Portland, OR, 97201, USA., Wilson DJ; Casey Eye Institute, Oregon Health & Science University, Portland, OR, 97201, USA., Anand-Apte B; Department of Ophthalmic Research, Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH, 44106, USA., Hurley JB; Department of Ophthalmology, University of Washington, Seattle, WA, 98109, USA; Department of Biochemistry, University of Washington, Seattle, WA, 98195, USA., Du J; Department of Ophthalmology, West Virginia University, Morgantown, WV, 26506, USA; Department of Biochemistry, West Virginia University, Morgantown, WV, 26506, USA. Electronic address: jianhai.du@wvumedicine.org., Chao JR; Department of Ophthalmology, University of Washington, Seattle, WA, 98109, USA. Electronic address: jrchao@uw.edu.

Publikováno v: Experimental eye research [Exp Eye Res] 2022 Feb; Vol. 215, pp. 108899. Date of Electronic Publication: 2021 Dec 17.

Reductive carboxylation is a major metabolic pathway in the retinal pigment epithelium.

Autor: Du J; Department of Biochemistry, University of Washington, Seattle, WA 98195; jianhai.du@wvumedicine.org jrchao@uw.edu.; Department of Ophthalmology, University of Washington, Seattle, WA 98109.; Department of Ophthalmology, West Virginia University, Morgantown, WV 26506., Yanagida A; Department of Ophthalmology, University of Washington, Seattle, WA 98109., Knight K; Department of Ophthalmology, University of Washington, Seattle, WA 98109., Engel AL; Department of Ophthalmology, University of Washington, Seattle, WA 98109., Vo AH; Department of Biochemistry, University of Washington, Seattle, WA 98195., Jankowski C; Department of Biochemistry, University of Washington, Seattle, WA 98195., Sadilek M; Department of Chemistry, University of Washington, Seattle, WA 98195., Tran VT; Department of Biochemistry, University of Washington, Seattle, WA 98195., Manson MA; Department of Ophthalmology, University of Washington, Seattle, WA 98109., Ramakrishnan A; Center for Blood Cancers and Oncology, St. David's South Austin Medical Center, Austin, TX 78704., Hurley JB; Department of Biochemistry, University of Washington, Seattle, WA 98195.; Department of Ophthalmology, University of Washington, Seattle, WA 98109., Chao JR; Department of Ophthalmology, University of Washington, Seattle, WA 98109; jianhai.du@wvumedicine.org jrchao@uw.edu.

Publikováno v: Proceedings of the National Academy of Sciences of the United States of America [Proc Natl Acad Sci U S A] 2016 Dec 20; Vol. 113 (51), pp. 14710-14715. Date of Electronic Publication: 2016 Dec 01.