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Hyperlipoproteinemia Type III MeSH Descriptor Data 2025
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.
Entry Term(s)
Autosomal Recessive Hypercholesterolemia
Broad Beta Disease
Dysbetalipoproteinemia
Dysbetalipoproteinemia, Familial
Familial Dysbetalipoproteinemia
Familial Hypercholesterolemia with Hyperlipemia
Hypercholesterolemia, Autosomal Recessive
Hyperlipoproteinemia, Broad-beta
Hyperlipoproteinemia, Type III
Previous Indexing
Hyperlipidemia/familial & genetic (1966-1979)
Public MeSH Note
1980; for AUTOSOMAL RECESSIVE HYPERCHOLESTEROLEMIA see HOMOZYGOUS FAMILIAL HYPERCHOLESTEROLEMIA 2022
History Note
1980; for AUTOSOMAL RECESSIVE HYPERCHOLESTEROLEMIA use HOMOZYGOUS FAMILIAL HYPERCHOLESTEROLEMIA 2022
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.