Aguie G A, Rader D J, Clavey V, Traber M G, Torpier G, Kayden H J, Fruchart J C, Brewer H B, Castro G
Serlia, Inserm U325, Institut Pasteur, Lille, France.
Atherosclerosis. 1995 Dec;118(2):183-91. doi: 10.1016/0021-9150(95)05605-x.
Abetalipoproteinemia (ABL) and homozygous hypobetalipoproteinemia (HBL) are inherited disorders which are classically characterized by progressive retinal and spinocerebellar disease, fat-soluble vitamin deficiency, and absence of apolipoprotein (apo) B from the plasma. Using immunoaffinity chromatography with an anti-apo B antiserum, we isolated apo B-containing lipoprotein (LpB) particles from the plasma of 4 ABL and 2 HBL patients. The LpB particles were characterized and compared with low density lipoprotein (LDL) and LpB isolated from normal plasma. The ABL/HBL LpB particles were similar in size and charge to normal LpB particles but were relatively enriched in several other apolipoproteins. They contained alpha-tocopherol in a ratio to cholesterol that was proportionately much higher than the very low ratio of alpha-tocopherol to cholesterol in plasma. They bound saturably to fibroblasts and were internalized and degraded similarly to LDL. Hence, the molecular defects in ABL and HBL permit the secretion of a very small number of apo B-containing lipoproteins which may be important for transport of alpha-tocopherol to peripheral tissues.
无β脂蛋白血症（ABL）和纯合子低β脂蛋白血症（HBL）是遗传性疾病，其典型特征为进行性视网膜和脊髓小脑疾病、脂溶性维生素缺乏以及血浆中缺乏载脂蛋白（apo）B。我们使用抗apo B抗血清通过免疫亲和层析从4例ABL患者和2例HBL患者的血浆中分离出含apo B的脂蛋白（LpB）颗粒。对LpB颗粒进行了表征，并与从正常血浆中分离出的低密度脂蛋白（LDL）和LpB进行了比较。ABL/HBL的LpB颗粒在大小和电荷方面与正常LpB颗粒相似，但在其他几种载脂蛋白中相对富集。它们所含α-生育酚与胆固醇的比例与血浆中α-生育酚与胆固醇的极低比例相比要高得多。它们可饱和地结合至成纤维细胞，并与LDL类似地被内化和降解。因此，ABL和HBL中的分子缺陷允许分泌极少量含apo B的脂蛋白，这对于将α-生育酚转运至外周组织可能很重要。
