Tangier disease: a disorder of intracellular membrane traffic

Journal Article · Sun Sep 01 00:00:00 EDT 1985 · Proc. Natl. Acad. Sci. U.S.A.; (United States)

DOI:https://doi.org/10.1073/pnas.82.18.6305· OSTI ID:6170251

Schmitz, G; Assmann, G; Robenek, H; Brennhausen, B

The interaction of human high density lipoproteins (HDL) with isolated human monocytes from control and Tangier patients was studied in tissue culture experiments. It was observed that normal monocytes, similar to mouse peritoneal macrophages, bind HDL to a cell surface receptor, internalize the bound HDL particles, transport the internalized HDL intracellularly through the cytoplasmic compartment without significant degradation, and ultimately resecrete intact HDL. The cellular interaction of Tangier monocytes with normal HDL was markedly different from control monocytes. HDL binding to Tangier monocytes was moderately increased and cell associated /sup 125/I-HDL was 6- to 10-fold enhanced in Tangier monocytes. The bulk of the internalized HDL, however, was detected in secondary lysosomes. These data suggest that the cellular metabolism of HDL is abnormal in Tangier monocytes. It is postulated that Tangier disease may be a disorder of intracellular membrane traffic in which HDL is diverted into the lysosomal compartment and degraded instead of being secreted through its regular transcellular route.

• Research Organization: Westfaelischen Wilhelms-Universitaet, Muenster, West Germany
• OSTI ID: 6170251
• Journal Information: Proc. Natl. Acad. Sci. U.S.A.; (United States), Vol. 82:18
• Country of Publication: United States
• Language: English

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