Plasma apolipoproteins in Tangier disease, as studied with two-dimensional electrophoresis.
Plasma apolipoproteins in Tangier disease, as studied with two-dimensional electrophoresis.
复制标题
通过二维电泳研究丹吉尔病中的血浆载脂蛋白。
DOI：
10.1093/clinchem/33.1.120
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发表时间：
1987
影响因子：
9.3
通讯作者：
G. Siest
中科院分区：
文献类型：
--
作者：
S. Visvikis;M. Dumon;J. Steinmetz;T. Manabe;M. Galteau;M. Clerc;G. Siest
丹吉尔病的特征是高密度脂蛋白及其主要蛋白质成分载脂蛋白（apo）A-I缺乏。我们使用高分辨率双向电泳检测了3名丹吉尔病患者、1名纯合子患者及其2名杂合子子女的主要血浆载脂蛋白（A-I、A-II、A-IV、E、C-II和C-III），并与健康受试者的模式进行了比较。在载脂蛋白A-I的同工蛋白分布中发现特征性异常，存在正常浓度的载脂蛋白A-I原，但其他载脂蛋白A-I同工蛋白的浓度显著降低。我们还发现载脂蛋白C-Ⅲ中迄今未描述的多肽异常：唾液酸化和非唾液酸化形式的载脂蛋白C-Ⅲ表现为具有相同等电点但分子量不同的双斑点。在其他血浆载脂蛋白的多肽分布中未检测到其他实质性差异。
Tangier disease is characterized by a deficiency of high-density lipoproteins and of their major protein constituent, apolipoprotein (apo) A-I. We used high-resolution two-dimensional electrophoresis to examine the principal plasma apolipoproteins (A-I, A-II, A-IV, E, C-II, and C-III) of three persons with Tangier disease, one homozygous patient and his two heterozygous children, comparing the patterns with those for healthy subjects. Characteristic abnormalities were found in the distribution of the isoproteins of apo A-I, there being a normal concentration of pro apo A-I but dramatically decreased concentrations of the other apo A-I isoproteins. We also found hitherto-undescribed polypeptide abnormalities in apo C-III: sialylated and nonsialylated forms of apo C-III appear as double spots having the same isoelectric points but different molecular masses. No other substantial difference was detected in the polypeptide distribution of the other plasma apolipoproteins.