tangier disease/protease

Cholesterol-loaded macrophage foam cells are a central component of atherosclerotic lesions. ABCA1, the defective molecule in Tangier disease, mediates the efflux of phospholipids and cholesterol from cells to apoA-I, reversing foam cell formation. In ABCA1, we identified a sequence rich in proline,

An enzymatic activity with releases p-nitroaniline from 3-carboxypropionyl-trialanine p-nitroanilide (Suc[Ala]3NA) was characterized in blood plasma of patients with Tangier disease. This activity results from the sequential action of a metalloendopeptidase (MP) and an aminopeptidase (AP). These

Tangier disease is a disorder characterized by low levels of apo-A-I and high density lipoproteins. The defect in Tangier disease is an abnormal A-I apolipo protein, designated apo-A- ITangier . In normal subjects, apo-A-I is secreted as proapo -A-I with subsequent extracellular conversion to mature

The low density lipoproteins (LDL) from patients with Tangier disease are enriched in triglycerides, 27% of LDL mass versus 7% for normal LDL. To study whether this unique LDL core lipid composition affects the surface disposition of apolipoprotein (apo) B-100, we analyzed the LDL by protease

Cholesterol-loaded macrophage foam cells are a central component of atherosclerotic lesions. ATP binding cassette transporter A1 (ABCA1), the defective molecule in Tangier disease, mediates the efflux of phospholipid and cholesterol from cells to apolipoprotein A-I (apoA-I), reversing foam cell