Holmquist L, Carlson L A
Acta Med Scand. 1987;221(3):283-9. doi: 10.1111/j.0954-6820.1987.tb00895.x.
Isolated high density lipoprotein (HDL) from the two living fish eye disease patients have been incubated in vitro with autologous lipoprotein depleted plasma or with lipoprotein depleted plasma from domestic pig (Sus domesticus), with and without the presence of LCAT inhibitor for 24 hours at 0 and 37 degrees C. The lecithin:cholesterol acyltransferase (LCAT) activity in lipoprotein depleted pig plasma increased the abnormally low cholesteryl ester content of the fish eye disease HDL particles from about 20 to 100% and increased their exceptionally small mean particle size, probably by particle fusion, to a range which is representative of normal HDL3. Both esterification and particle enlargement were totally blocked by the LCAT inhibitor. Incubation of concentrated fish eye disease HDL with autologous lipoprotein depleted plasma for 24 hours at 37 degrees C resulted in a small increase in its cholesteryl ester percentage to 37%, without affecting the apparent HDL particle size. This finding confirms a deficiency of HDL lecithin:cholesterol acyltransferase activity (alpha-LCAT) in fish eye disease. The observed normalization of both HDL cholesteryl ester percentage and particle size by lipoprotein depleted pig plasma which contains virtually no cholesteryl ester transfer activity indicates that the latter is not a requisite for esterification of the free cholesterol of fish eye disease HDL.
从两名患有鱼眼病的在世患者身上分离出的高密度脂蛋白（HDL），已在体外与自体脂蛋白缺乏血浆或家猪（Sus domesticus）的脂蛋白缺乏血浆一起孵育，分别在有和没有卵磷脂胆固醇酰基转移酶（LCAT）抑制剂存在的情况下，于0和37摄氏度孵育24小时。脂蛋白缺乏的猪血浆中的卵磷脂：胆固醇酰基转移酶（LCAT）活性，使鱼眼病HDL颗粒中异常低的胆固醇酯含量从约20%增加到100%，并使它们异常小的平均颗粒大小增加，可能是通过颗粒融合，增加到代表正常HDL3的范围。酯化和颗粒增大均被LCAT抑制剂完全阻断。将浓缩的鱼眼病HDL与自体脂蛋白缺乏血浆在37摄氏度孵育24小时，导致其胆固醇酯百分比小幅增加至37%，而不影响HDL颗粒的表观大小。这一发现证实了鱼眼病中HDL卵磷脂：胆固醇酰基转移酶活性（α-LCAT）的缺乏。含有几乎没有胆固醇酯转移活性的脂蛋白缺乏猪血浆，使HDL胆固醇酯百分比和颗粒大小均恢复正常，这表明后者不是鱼眼病HDL游离胆固醇酯化的必要条件。
