citation needed] Type I hyperlipoproteinemia exists in several forms: Lipoprotein lipase deficiency (type Ia), due to a deficiency of lipoprotein lipase (LPL) or altered apolipoprotein C2, resulting in elevated chylomicrons, the particles that transfer fatty acids from the digestive tract to the liver Familial apoprotein CII deficiency (type Ib), a condition caused by a lack of lipoprotein lipase activator. (wikipedia.org)
In the peripheral tissues, particularly adipose and muscle tissue, VLDL is cleaved by lipoprotein lipase (LPL), extracting most of the triglycerides and forming an IDL that contains apoproteins B-100 and E. IDL can be taken up by the liver through the LDL receptor, or it can be converted to the cholesterol-rich LDL that contains apoprotein B-100. (medscape.com)
Familial lipoprotein lipase deficiency is an example of a primary disorder in which a deficiency of lipoprotein lipase in tissue leads to a type I pattern of hyperlipidemia, with a massive accumulation of chylomicrons in the plasma. (medscape.com)
Inactivity of lipoprotein lipase (LPL) plays the predominant role in the development of familial hypertriglyceridemia. (mdwiki.org)
VLDL3
The liver secretes the triglyceride-rich VLDL that contains apoproteins B-100, C-II, and E into the circulation. (medscape.com)
HDL particles that contain apoproteins A-I and A-II interact with other lipoproteins, particularly VLDL and LDL, through lipolysis and the action of lecithin cholesterol acyltransferase (LCAT) enzyme. (medscape.com)
Familial hypertriglyceridemia (type IV familial dyslipidemia) is a genetic disorder characterized by the liver overproducing very-low-density lipoproteins (VLDL). (mdwiki.org)
The endogenous lipoprotein pathway refers to lipoproteins and apoproteins that are synthesized in tissues other than the intestines, predominantly in the liver. (medscape.com)
Hyperlipoproteinemia1
Fatty liverhepatitis and type 5 hyperlipoproteinemia in juvenile diabetes mellitus. (nih.gov)
Familial LDL receptor deficiency and familial defective apoprotein B-100 are examples of primary defects that can lead to the accumulation of LDL, which corresponds to a type IIa pattern of hyperlipidemia. (medscape.com)
Familial hypertriglyceridemia is considered a type IV familial dyslipidemia it is distinguished from other dyslipidemias based on the individual's lipid profile. (mdwiki.org)
Genetic1
Hyperlipidemias may basically be classified as either familial (also called primary) when caused by specific genetic abnormalities or acquired (also called secondary) when resulting from another underlying disorder that leads to alterations in plasma lipid and lipoprotein metabolism. (wikipedia.org)
Mutation1
[4] One of the most common mutations implicated in the development of familial hypertriglyceridemia is a heterozygous inactivating mutation of the LPL gene. (mdwiki.org)
Primarily2
LDL is removed from the circulation primarily by the liver through the LDL receptor. (medscape.com)
Treatment for familial hypertriglyceridemia should focus primarily on reducing serum triglyceride levels. (mdwiki.org)
Significantly1
Familial hypertriglyceridemia separates itself from other dyslipidemias with significantly high triglycerides and low HDL levels. (mdwiki.org)
Primary1
There are other varying secondary causes of pancreatitis that can further contribute to the primary scenario of pancreatitis related to familial hypertriglyceridemia. (mdwiki.org)
Defective2
Familial LDL receptor deficiency and familial defective apoprotein B-100 are examples of primary defects that can lead to the accumulation of LDL, which corresponds to a type IIa pattern of hyperlipidemia. (medscape.com)
Typing, too, had as its purpose the better segregation of human mutants;when a defective gene fails to produce a functioning apoprotein, a unique opportunity to understand the function and importance of that protein may present itself. (nih.gov)