Article
A Drosophila model for the Zellweger spectrum of peroxisome biogenesis disorders.
Disease models & mechanisms - 1 Sept 2011
Mast Fred D, Li Jing, Virk Maninder K, Hughes Sarah C, Simmonds Andrew J, Rachubinski Richard A
Abstract excerpt
Human peroxisome biogenesis disorders are lethal genetic diseases in which abnormal peroxisome assembly compromises overall peroxisome and cellular function. Peroxisomes are ubiquitous membrane-bound organelles involved in several important biochemical processes, notably lipid metabolism and the use of reactive oxygen species for detoxification. Using cultured cells, we systematically characterized the peroxisome...
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