trappc11 is required for protein glycosylation in zebrafish and humans

C. DeRossi,Ana M. Vacaru,Ruhina Rafiq,A. Cinaroglu,D. Imrie,Shikha Nayar,A. Baryshnikova,Miroslav P. Milev,Daniela Stanga,Dhara Kadakia,Ningguo Gao,Jaime Chu,H. Freeze,M. Lehrman,M. Sacher,K. Sadler

Published 2016 in Molecular Biology of the Cell

ABSTRACT

The trappc11 mutation in zebrafish causes a stressed UPR, leading to fatty liver disease. This phenotype is not due to a trafficking defect but instead results from N-glycosylation defects. Hypoglycosylation and lipid accumulation are also found in patient cells with knockdown or mutated TRAPPC11 genes, suggesting a common etiology with zebrafish.

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