Effects of plasma infusion on plasma lipids, apoproteins and plasma enzyme activities in familial lecithin: cholesterol acyltransferase deficiency.

Abstract:

:The siblings presented here are the third family found in Japan with familial LCAT deficiency. Their post-heparin plasma lipoprotein lipase and hepatic triglyceride lipase activities were measured selectively by an immunochemical method. Plasma triglyceride levels were elevated, and post-heparin plasma lipoprotein lipase was decreased only in a patient with nephropathy, while hepatic triglyceride lipase activities were within reference limits in both patients. The plasma concentrations of apo A-I, apo A-II, and apo B were reduced in both patients. On the other hand, the plasma concentration of apo E was markedly increased. Enzyme replacement therapy by plasma transfusion in the propositus resulted in marked improvement of deranged compositions of triglyceride-rich lipoproteins. Also, improvement of the plasma apo E concentration was demonstrated, while the improvement of post-heparin lipase did not occur. These results suggest that LCAT may play an important physiological role in triglyceride metabolism as well as in cholesterol metabolism.

journal_name

Eur J Clin Invest

authors

Murayama N,Asano Y,Kato K,Sakamoto Y,Hosoda S,Yamada N,Kodama T,Murase T,Akanuma Y

doi

10.1111/j.1365-2362.1984.tb02100.x

subject

Has Abstract

pub_date

1984-04-01 00:00:00

pages

122-9

issue

2

eissn

0014-2972

issn

1365-2362

journal_volume

14

pub_type

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