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Lipase deficiencies
Author(s) -
Greten H.,
Beil F. U.
Publication year - 1988
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01800568
Subject(s) - chylomicron , lipoprotein lipase , very low density lipoprotein , apolipoprotein c2 , hepatic lipase , lipase , triglyceride , apolipoprotein b , endocrinology , medicine , lipoprotein , triglyceride lipase , chemistry , enzyme , biochemistry , biology , cholesterol
Summary Two enzymes, lipoprotein lipase and hepatic triglyceride lipase, are involved in the hydrolysis of triglycerides from chylomicrons and very low density lipoprotein (VLDL). Lipoprotein lipase has an absolute requirement for apolipoprotein CII for activity. Three inborn errors of metabolism which give rise to hypertriglyceridaemia have been described. The biochemical and clinical aspects of these disorders, lipoprotein lipase deficiency (familial type I hyperlipoproteinaemia), hepatic triglyceride lipase deficiency and apo‐CII deficiency are discussed.