The A B Cs of Tangier Disease

Main Category: Cholesterol
Article Date: 13 Apr 2005 - 3:00 PDT

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High levels of HDL, the "good" cholesterol, are associated with low cardiovascular disease risk. Patients with Tangier disease have very low levels of HDL due to mutations in a protein called ABCA1. Exactly how ABCA1 controls HDL formation was not clear.

In a study appearing online on April 7 in advance of the print publication of the May 1 issue of the Journal of Clinical Investigation, John Parks and colleagues from Wake Forest University describe a new mouse model that can be used to make liver-specific deletions of ABCA1.

The mice have an 80% decrease in plasma HDL levels. This is the first proof that liver ABCA1 is critically important for normal HDL metabolism and may explain why normal HDL is broken down extensively in Tangier patients.

TITLE: Targeted inactivation of hepatic Abca1 causes profound hypoalphalipoproteinemia and kidney hypercatabolism of apoA-I

AUTHOR CONTACT:
John S. Parks
Wake Forest University School of Medicine, Winston-Salem, NC USA
Phone: (336) 716-2145; Fax: (336) 716-6279; E-mail: jparks@wfubmc.edu

View the PDF of this article at: the-jci.org/article.php?id=23915

Contact: Stacie Bloom
press_releases@the-jci.org
212 342-4159
Journal of Clinical Investigation
http://www.jci.org

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