Other terms
Dysbetalipoproteinemia,Dysbetalipoproteinemia, Familial,Familial Hypercholesterolemia with Hyperlipemia,Hyperlipoproteinemia, Broad-beta,Broad-beta Hyperlipoproteinemia,Hyperlipoproteinemia, Broad beta,Broad Beta Disease,Familial Dysbetalipoproteinemia
Description
Hyperlipoproteinemia Type III: An autosomal recessively inherited disorder characterized by the accumulation of intermediate-density lipoprotein (IDL or broad-beta-lipoprotein). IDL has a CHOLESTEROL to TRIGLYCERIDES ratio greater than that of VERY-LOW-DENSITY LIPOPROTEINS. This disorder is due to mutation of APOLIPOPROTEINS E, a receptor-binding component of VLDL and CHYLOMICRONS, resulting in their reduced clearance and high plasma levels of both cholesterol and triglycerides.
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