Hyperlipoproteinemia Type III

Synonyms

Broad Beta Disease

Broad-beta Hyperlipoproteinemia

Dysbetalipoproteinemia

Dysbetalipoproteinemia, Familial

Familial Dysbetalipoproteinemia

Familial Hypercholesterolemia with Hyperlipemia

Hyperlipoproteinemia, Broad beta

Hyperlipoproteinemia, Broad-beta

Hyperlipoproteinemia, Type III

Hyperlipoproteinemias, Type III

Type III Hyperlipoproteinemia

Type III Hyperlipoproteinemias

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.