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J. Lipid Res.
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A more recent version of this article appeared on February 1, 2009

Papers In Press, published online ahead of print September 24, 2008
J. Lipid Res., doi:10.1194/jlr.M800415-JLR200
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Submitted on August 5, 2008
Revised on September 17, 2008
Accepted on September 24, 2008

TNF-alpha plays a role in hepatocyte apoptosis in Niemann Pick type C liver disease

Victoria M. Rimkunas, Mark J. Graham, Rosanne M. Crooke, and Laura Liscum

Physiology, Tufts University, Boston, MA 02111

Corresponding Author: victoria.rimkunas{at}gmail.com

Niemann Pick type C (NPC) is a fatal autosomal recessive lysosomal storage disease clinically characterized by neurodegeneration and liver disease. Heterogeneous mutations in the NPC1 and NPC2 genes cause impaired egress of free cholesterol from lysosomes leading to accumulation of cholesterol and glycosphingolipids. Key features of NPC liver disease include hepatic apoptosis, inflammation and fibrosis. It is unclear what signaling events regulate these disease processes in NPC. We hypothesize that tumor necrosis factor a (TNF-a), which is involved in both proinflammatory and apoptotic signaling cascades, is a key mediator of inflammation, apoptosis and fibrosis in NPC liver disease. In this study, we evaluated the role of TNF-a signaling in NPC liver disease by utilizing NPC1 specific antisense oligonucleotides to knockdown NPC1 expression in control and TNF-a knockout mice. In the absence of TNF-a, NPC1 knockdown produced liver disease with significantly less inflammation, apoptosis and fibrosis.


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N. L. Sayre, V. M. Rimkunas, M. J. Graham, R. M. Crooke, and L. Liscum
Recovery from liver disease in a Niemann-Pick type C mouse model
J. Lipid Res., August 1, 2010; 51(8): 2372 - 2383.
[Abstract] [Full Text] [PDF]


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