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Torsins Are Essential Regulators of Cellular Lipid Metabolism

Journal Contribution - Journal Article

Torsins are developmentally essential AAA+ proteins,and mutation of human torsinA causes theneurological disease DYT1 dystonia. They localizein the ER membranes, but their cellular function remainsunclear. We now show that dTorsin is requiredin Drosophila adipose tissue, where it suppresses triglyceridelevels, promotes cell growth, and elevatesmembrane lipid content.We also see that human torsinAat the inner nuclear membrane is associatedwith membrane expansion and elevated cellular lipidcontent. Furthermore, the key lipid metabolizingenzyme, lipin, is mislocalized in dTorsin-KO cells,and dTorsin increases levels of the lipin substrate,phosphatidate, and reduces the product, diacylglycerol.Finally, genetic suppression of dLipin rescuesdTorsin-KO defects, including adipose cell size, animalgrowth, and survival. These findings identify thattorsins are essential regulators of cellular lipid metabolismand implicate disturbed lipid biology in childhood-onset DYT1 dystonia.
Journal: Developmental Cell
ISSN: 1534-5807
Issue: 3
Volume: 38
Pages: 235 - 247
BOF-keylabel:yes
IOF-keylabel:yes
BOF-publication weight:10
CSS-citation score:2
Authors:International
Authors from:Higher Education
Accessibility:Closed