Taskinen M R, Glueck C J, Kashyap M L, Srivastava L S, Hynd B A, Perisutti G, Robinson K, Kinnunen P J, Kuusi T
Atherosclerosis. 1980 Oct;37(2):247-56. doi: 10.1016/0021-9150(80)90010-6.
Post-heparin lipoprotein lipase (PH-LPL)-high density lipoprotein cholesterol (HDL-C) interrrelationships were assessed in 9 subjects with documented familial hyperalphalipoproteinemia (FHA) and in 8 controls to focus on potential biochemical etiologies of FHA and relationships of HDL-C to triglyceride hydrolysis and PH-LPL. FHA subjects had mean HDL-C and HDL2-C levels > twice controls; their PH-LPL levels (mean +/- SEM) (3.14 +/- 2.3 mumol FFA/h/ml) were also > twice that of controls (15.0 +/- 1.6) (P < 0.01), but post-heparin hepatic lipase levels (PH-HL) in the FHA and control subjects did not differ (18.1 +/- 1.6 vs 26.6 +/- 4.3, P > 0.1). For all subjects (FHA and controls) PH-LPL was positively correlated with HDL-C (r = 0.79, P < 0.01) and with HDL2-C (r = 0.90, P < 0.01), but not with HDL3-C (r = --0.02). There were no significant PH-HL and HDL-C interrelationships, P > 0.1. The amount of apo CII (the primary activator of PH-LPL) in HDL2 was greater in the FHA (mean +/- SEM) (16.1 +/- 2.5 microgram/ml plasma) than in control subjects (4.7 +/- 0.9, P < 0.01). There were strong positive correlations between HDL2 apo CII and both PH-LPL (r = 0.79, P < 0.01) and HDL2-C (r = 0.80, P < 0.01). Apo CII as a percentage of HDL2 protein was higher in FHA than control subjects (mean +/- SEM) (1.2 +/- 0.3% vs 0.5 +/- 0.2%, P < 0.01). Apo CII as a percentage of HDL3 protein was similar in FHA and control subjects. We postulate that increased turnover rate of triglyceride-rich lipoproteins due to high LPL activity may be an important factor leading to the elevation of HDL-C in FHA. The highly significant positive correlation between HDL2-C and PH-LPL provides strong clinical evidence for the theory that HDL2 is formed during the hydrolysis of triglycceride-rich lipoproteins. The high concentration of HDL2 apo CII in FHA subjects may be caused by increased catabolism of triglyceride-rich lipoproteins in the presence of high endothelial LPL, with transfer of apo CII from very low to high density lipoproteins.
对9名确诊为家族性高α脂蛋白血症(FHA)的受试者和8名对照者进行了肝素后脂蛋白脂肪酶(PH-LPL)与高密度脂蛋白胆固醇(HDL-C)的相互关系评估,以关注FHA潜在的生化病因以及HDL-C与甘油三酯水解和PH-LPL的关系。FHA受试者的平均HDL-C和HDL2-C水平是对照者的两倍以上;他们的PH-LPL水平(均值±标准误)(3.14±2.3μmol游离脂肪酸/小时/毫升)也高于对照者的两倍(15.0±1.6)(P<0.01),但FHA受试者和对照者的肝素后肝脂肪酶水平(PH-HL)无差异(18.1±1.6对26.6±4.3,P>0.1)。对于所有受试者(FHA和对照者),PH-LPL与HDL-C呈正相关(r = 0.79,P<0.01),与HDL2-C呈正相关(r = 0.90,P<0.01),但与HDL3-C无相关性(r = -0.02)。PH-HL与HDL-C之间无显著的相互关系,P>0.1。FHA受试者HDL2中载脂蛋白CII(PH-LPL的主要激活剂)的含量(均值±标准误)(16.1±2.5μg/毫升血浆)高于对照受试者(4.7±0.9,P<0.01)。HDL2载脂蛋白CII与PH-LPL(r = 0.79,P<0.01)和HDL2-C(r = 0.80,P<0.01)均呈强正相关。FHA受试者中载脂蛋白CII占HDL2蛋白的百分比高于对照受试者(均值±标准误)(1.2±0.3%对0.5±0.2%,P<0.01)。FHA受试者和对照受试者中载脂蛋白CII占HDL3蛋白的百分比相似。我们推测,由于高LPL活性导致富含甘油三酯的脂蛋白周转率增加可能是导致FHA中HDL-C升高的一个重要因素。HDL2-C与PH-LPL之间高度显著的正相关为HDL2在富含甘油三酯的脂蛋白水解过程中形成这一理论提供了有力的临床证据。FHA受试者中HDL2载脂蛋白CII的高浓度可能是由于在内皮LPL水平高的情况下富含甘油三酯的脂蛋白分解代谢增加,载脂蛋白CII从极低密度脂蛋白转移到高密度脂蛋白所致。