Characterization of an abnormal species of apolipoprotein B, apolipoprotein B-37, associated with familial hypobetalipoproteinemia.

Characterization of an abnormal species of apolipoprotein B, apolipoprotein B-37, associated with familial hypobetalipoproteinemia.
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与家族性低β脂蛋白血症相关的载脂蛋白 B 异常种类(载脂蛋白 B-37)的表征。

DOI:
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发表时间:
1987
影响因子:
15.9
通讯作者:
J. Witztum
J. Witztum
中科院分区:
医学1区
文献类型:
--
作者:
S. Young;S. Bertics;L. Curtiss;J. Witztum

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斯坦伯格及其同事先前描述了一个具有正常甘油三酯血症性低β脂蛋白血症的独特家族(1979年,《临床研究杂志》64:292 - 301)。在对这个家族的再次检查中,我们在该家族多个成员的血浆脂蛋白中发现了一种异常的载脂蛋白(apo)B种类,即apo B - 37(分子量203,000)。在患病个体中,apo B - 37与正常的apo B种类apo B - 100和apo B - 48一起存在于极低密度脂蛋白中。高密度脂蛋白(HDL)也含有apo B - 37,但没有其他apo B种类。apo B - 37的前13个氨基末端氨基酸与正常的apo B - 100相同。我们使用了一组18种不同的apo B特异性单克隆抗体以及对apo B - 37和apo B - 100的凝血酶裂解产物具有特异性的多克隆抗血清,来绘制apo B - 37相对于apo B - 100、apo B - 48以及apo B - 100的凝血酶裂解产物的图谱。这些免疫化学研究的结果表明,apo B - 37仅包含apo B - 100的氨基末端结构域。在患病个体中,血浆中的大部分apo B - 37包含在HDL密度组分中。在该组分中,apo B - 37存在于离散的脂蛋白颗粒上,称为Lp - B37,其性质不同于含有apo A - I的正常HDL颗粒。本报告首次记录了人类中存在一种异常的apo B种类。对apo B - 37和含有apo B - 37的脂蛋白颗粒的进一步研究应能增进对apo B结构和功能的理解。
Steinberg and colleagues have previously described a unique kindred with normotriglyceridemic hypobetalipoproteinemia (1979. J. Clin. Invest. 64:292-301). In a reexamination of this kindred, we found an abnormal apolipoprotein (apo) B species, apo B-37 (203,000 mol wt), in the plasma lipoproteins of multiple members of the kindred. In affected individuals apo B-37 was found in very low density lipoproteins, along with the normal apo B species, apo B-100 and apo B-48. High density lipoproteins (HDL) also contained apo B-37, but no other apo B species. The first 13 amino-terminal amino acids of apo B-37 were identical to those of normal apo B-100. We utilized a panel of 18 different apo B-specific monoclonal antibodies and polyclonal antisera specific for apo B-37 and the thrombin cleavage products of apo B-100 to map apo B-37 in relation to apo B-100, apo B-48, and the thrombin cleavage products of apo B-100. The results of those immunochemical studies indicated that apo B-37 contains only amino-terminal domains of apo B-100. In affected individuals, the majority of apo B-37 in plasma was contained in the HDL density fraction. Within that fraction apo B-37 was found on discrete lipoprotein particles, termed Lp-B37, that had properties distinct from normal HDL particles containing apo A-I. This report documents for the first time the existence of an abnormal apo B species in humans. Further study of apo B-37 and lipoprotein particles containing apo B-37 should lead to an improved understanding of apo B structure and function.
DOI: 10.1016/0005-2760(81)90253-8
发表时间: 1981-01-01
期刊: BIOCHIMICA ET BIOPHYSICA ACTA
影响因子: --
作者:
BLANCHE, PJ;GONG, EL;NICHOLS, AV
通讯作者: NICHOLS, AV
DOI: 10.1126/science.3513311
发表时间: 1986-04-04
期刊: SCIENCE
影响因子: 56.9
作者:
BROWN, MS;GOLDSTEIN, JL
通讯作者: GOLDSTEIN, JL
组织和血浆激肽释放酶降解人血浆低密度脂蛋白的载脂蛋白 B-100。
DOI: --
发表时间: 1984
期刊: The Journal of biological chemistry
影响因子: --
作者:
Cardin,AD;Witt,KR;Chao,J;Margolius,HS;Donaldson,VH;Jackson,RL
通讯作者: Jackson,RL
DOI: --
发表时间: 1985
期刊: The Journal of biological chemistry
影响因子: --
作者:
Curtiss,LK;Edgington,TS
通讯作者: Edgington,TS
DOI: 10.1073/pnas.81.12.3883
发表时间: 1984-01-01
期刊: PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES
影响因子: --
作者:
STEINBRECHER, UP;PARTHASARATHY, S;STEINBERG, D
通讯作者: STEINBERG, D