Article
Single Mutation in the NFU1 Gene Metabolically Reprograms Pulmonary Artery Smooth Muscle Cells.
Arteriosclerosis, thrombosis, and vascular biology - 1 Feb 2021
James Joel, Zemskova Marina, Eccles Cody A, Varghese Mathews V, Niihori Maki, Barker Natalie K, Luo Moulun, Mandarino Lawrence J, Langlais Paul R, Rafikova Olga, Rafikov Ruslan
Abstract excerpt
OBJECTIVE: NFU1 is a mitochondrial iron-sulfur scaffold protein, involved in iron-sulfur assembly and transfer to complex II and LAS (lipoic acid synthase). Patients with the point mutation NFU1G208C and CRISPR/CAS9 (clustered regularly interspaced short palindromic repeats/clustered regularly interspaced short palindromic repeat-associated 9)-generated rats develop mitochondrial dysfunction leading to pulmonary...
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