Hepatic Lipase Deficiency in a Middle-Eastern/Arabic Male∗
نویسندگان
چکیده
منابع مشابه
Plasma lipoproteins in familial hepatic lipase deficiency.
We have studied the lipoproteins, apolipoproteins, and postheparin lipase activities in an extended pedigree with familial hepatic lipase deficiency. A deficiency of hepatic lipase was found in three of five brothers and in one of their children. Triglyceride enrichment of low density and high density lipoproteins was identified as the constitutive phenotype. beta-very low density lipoprotein w...
متن کاملLarge buoyant LDL-like particles in hepatic lipase deficiency.
Hepatic lipase (HL) is thought to play a role in processing very low density lipoprotein to low density lipoprotein (LDL). To analyze the relationship between HL and LDL, the density, size, and chemical composition of LDL isolated from 18 normal subjects and from three subjects with reduced or absent levels of HL activity were compared. In an HL-deficient subject, the major peak of apoprotein (...
متن کاملHepatic lipase deficiency. Clinical, biochemical, and molecular genetic characteristics.
Hepatic lipase (HL) is an important enzyme in the metabolism of triglyceride-rich lipoproteins and high density lipoproteins. The clinical syndrome of HL deficiency is rare and difficult to identify. We studied carriers of mutant HL to ascertain whether there are distinctive clinical and/or biochemical characteristics of the heterozygous state. In an Ontario kindred, compound heterozygosity for...
متن کاملChimeras of Hepatic Lipase and Lipoprotein Lipase
Chimeric molecules between human lipoprotein lipase (LPL) and rat hepatic lipase (HL) were used to identify structural elements responsible for functional differences. Based on the close sequence homology with pancreatic lipase, both LPL and HL are believed to have a two-domain structure composed of an aminoterminal (NHz-terminal) domain containing the catalytic Ser-His-Asp triad and a smaller ...
متن کاملCongenital Pancreatic Lipase Deficiency.
The purpose of this communication is to record two families, each consisting of two children. The four children have presented an identical clinical picture associated with a congenital severe reduction of pancreatic lipase, and have responded to persistent treatment with lipase given as pancreatin. A brief reference was made to Case 1 in the Charles West Lecture, delivered before the Royal Col...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Journal of Clinical Lipidology
سال: 2010
ISSN: 1933-2874
DOI: 10.1016/j.jacl.2010.03.065