Article
A precursor-inducible zebrafish model of acute protoporphyria with hepatic protein aggregation and multiorganelle stress.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 May 2016
Elenbaas Jared S, Maitra Dhiman, Liu Yang, Lentz Stephen I, Nelson Bradley, Hoenerhoff Mark J, Shavit Jordan A, Omary M Bishr
Abstract excerpt
Protoporphyria is a metabolic disease that causes excess production of protoporphyrin IX (PP-IX), the final biosynthetic precursor to heme. Hepatic PP-IX accumulation may lead to end-stage liver disease. We tested the hypothesis that systemic administration of porphyrin precursors to zebrafish larvae results in protoporphyrin accumulation and a reproducible nongenetic porphyria model. Retro-orbital infusion of...
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