Fatty liver in familial hypobetalipoproteinemia: triglyceride assembly into VLDL particles is affected by the extent of hepatic steatosis
Fatty liver in familial hypobetalipoproteinemia: triglyceride assembly into VLDL particles is affected by the extent of hepatic steatosis
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DOI：
10.1194/jlr.m200342-jlr200
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发表时间：
2003-03-01
影响因子：
6.5
通讯作者：
Ackerman, J
中科院分区：
文献类型：
--
作者：
Schonfeld, G;Patterson, BW;Ackerman, J
家族性低β脂蛋白血症（FHBL）受试者可能会患上脂肪肝。通过磁共振波谱 (MRS) 对具有 6 种不同载脂蛋白 B (apoB) 截短（apoB-4 至 apoB-89）的 21 个 FHBL 和 14 个对照进行了肝脏脂肪评估。肝脏脂肪百分比为 16.7 +/- 11.5 和 3.3 +/- 2.9（平均值 +/- SD）（P = 0.001）。肝脏脂肪百分比与体重指数、腰围和2小时糖耐量试验胰岛素曲线下面积呈正相关，表明肥胖可能影响两组肝脏脂肪堆积的严重程度。尽管肝脏脂肪百分比存在 5 倍差异，但肥胖和胰岛素指数的平均值相似。因此，对于相似程度的肥胖，FHBL 受试者的肝脏脂肪较多。 VLDL-甘油三酯（TG）-脂肪酸来自血浆和非血浆来源（肝脏和内脏组织）。为了评估每种药物的相对贡献，13 名 FHBL 受试者和 11 名对照者被注射[H-2(2)]棕榈酸酯超过 12 小时。通过质谱法测定血浆游离棕榈酸酯和VLDL-TG-棕榈酸酯的同位素富集。非血浆来源在 FHBL 中占 51 +/- 15%，在对照中占 37 +/- 13%（P = 0.02）。 FHBL 受试者的肝脏脂肪百分比和肝脏中 VLDL-TG-棕榈酸酯百分比的相关性为 r = 0.89 (P = 0.0001)，对照者为 r = 0.69 (P = 0.01)。jlr 因此，apoB 截短产生的突变会导致脂肪肝和 VLDL-TG 组装的改变。
Familial hypobetalipoproteinemia (FHBL) subjects may develop fatty liver. Liver fat was assessed in 21 FHBL with six different apolipoprotein B (apoB) truncations (apoB-4 to apoB-89) and 14 controls by magnetic resonance spectroscopy (MRS). Liver fat percentages were 16.7 +/- 11.5 and 3.3 +/- 2.9 (mean +/- SD) (P = 0.001). Liver fat percentage was positively correlated with body mass index, waist circumference, and areas under the insulin curves of 2 h glucose tolerance tests, suggesting that obesity may affect the severity of liver fat accumulation in both groups. Despite 5-fold differences in liver fat percentage, mean values for obesity and insulin indexes were similar. Thus, for similar degrees of obesity, FHBL subjects have more hepatic fat. VLDL-triglyceride (TG)-fatty acids arise from plasma and nonplasma sources (liver and splanchnic tissues). To assess the relative contributions of each, [H-2(2)]palmitate was infused over 12 h in 13 FHBL subjects and 11 controls. Isotopic enrichment of plasma free palmitate and VLDL-TG-palmitate was determined by mass spectrometry. Non-plasma sources contributed 51 +/- 15% in FHBL and 37 +/- 13% in controls (P = 0.02). Correlations of liver fat percentage and percent VLDL-TG-palmitate from liver were r = 0.89 (P = 0.0001) for FHBL subjects and r = 0.69 (P = 0.01) for controls.jlr Thus, apoB truncation-producing mutations result in fatty liver and in altered assembly of VLDL-TG.