CHYLOMICRONEMIA SYNDROME DUE TO LIPOPROTEIN-LIPASE (LPL) DEFICIENCY - A POSSIBLE CAUSE OF METABOLIC EMERGENCY

被引：0

作者：

FERRARIS, S ^{[1
]}

SPADA, M ^{[1
]}

FERRERO, GB ^{[1
]}

DOMPE, C ^{[1
]}

PONZONE, A ^{[1
]}

机构：

[1] PEDIAT CLIN,I-10126 TURIN,ITALY

来源：

RIVISTA ITALIANA DI PEDIATRIA-ITALIAN JOURNAL OF PEDIATRICS | 1995年 / 21卷 / 02期

关键词：

HYPERTRIGLYCERIDEMIA; CHYLOMICRONEMIA SYNDROME; LPL DEFICIENCY;

D O I：

暂无

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Lipoprotein lipase (LPL) plays a major role in the regulation of lipid lipid metabolism. Its primary function is the hydrolysis of triglyceride core of chylomicrons and very low density lipoproteins. In addition, LPL activity allows the maturation of several classes of lipoproteins. Impaired lipolisys due to LPL deficiency causes hypertriglyceridemia and chylomicronemia syndrome. Two primary defects of LPL activity are known, the familial deficiencies of LPL and of apo CII, the natural cofactor of LPL. Circulating inhibitor of LPL has also been reported. Two paradigmatic cases of chylomicronemia syndrome due to familial LPL deficiency are described.

引用

页码：247 / 252

页数：6