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Medical Definition of Tangier disease
1. A familial lipoprotein deficiency caused by a decreased synthesis and increased catabolism of the apolipoprotein components a-l and a-II (apo a-I and apo a-II) of high-density lipoproteins (hdl) (lipoproteins, hdl). Hdl is absent from plasma, and the other lipoproteins are abnormal. Cholesteryl esters accumulate in the reticuloendothelial cells. It is also called analphalipoproteinaemia and familial high-density lipoprotein (hdl) deficiency. It was named after the chesapeake bay island where the first cases were identified. (12 Dec 1998)