Layman D L, Jelen B J, Illingworth D R
Proc Natl Acad Sci U S A. 1980 Mar;77(3):1511-5. doi: 10.1073/pnas.77.3.1511.
Serum from two patients with abetalipoproteinemia, a rare disorder of lipid metabolism characterized by the absence of chylomicrons and very low density and low density lipoproteins, did not stimulate the proliferation and growth of human smooth muscle cells or dermal fibroblasts in vitro as effectively as normal serum. The growth-promoting activity of this serum was comparable to that observed for lipoprotein-deficient plasma from normolipidemic subjects. Although the mitogenic effect of abetalipoproteinemic serum was improved with supplementation of low density lipoproteins, it was still about half the activity achieved with normal serum. However, the growth-promoting activity of this serum was completely restored to normal levels after addition of a lysate of normal platelets. In contrast, the mitogenic activity of lipoprotein-deficient plasma remained unchanged after addition of a lysate from abetalipoproteinemic platelets, whereas a similar supplementation of normal platelets completely restored its growth-promoting activity to normal. Thus, the inability of abetalipoproteinemic serum to promote growth appears to be due both to a deficiency of a platelet-releasable growth factor(s) and to the absence of serum lipoproteins.
两名患有无β脂蛋白血症(一种罕见的脂质代谢紊乱疾病,其特征为缺乏乳糜微粒以及极低密度脂蛋白和低密度脂蛋白)患者的血清,在体外对人平滑肌细胞或真皮成纤维细胞增殖和生长的刺激效果不如正常血清。这种血清的促生长活性与正常血脂个体的脂蛋白缺乏血浆所观察到的活性相当。尽管补充低密度脂蛋白后无β脂蛋白血症血清的促有丝分裂作用有所改善,但仍约为正常血清所达到活性的一半。然而,添加正常血小板裂解物后,这种血清的促生长活性完全恢复到正常水平。相反,添加无β脂蛋白血症血小板的裂解物后,脂蛋白缺乏血浆的促有丝分裂活性保持不变,而类似地补充正常血小板则将其促生长活性完全恢复到正常。因此,无β脂蛋白血症血清无法促进生长似乎既是由于血小板可释放生长因子的缺乏,也是由于血清脂蛋白的缺失。