Myelomatosis with Type III Hyperlipoproteinemia

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Pathogenesis of type III hyperlipoproteinemia (dysbetalipoproteinemia). Questions, quandaries, and paradoxes.

Type III hyperlipoproteinemia (HLP) is a genetic disorder characterized by accumulation of remnant lipoproteins in the plasma and development of premature atherosclerosis. Although receptor binding-defective forms of apolipoprotein (apo) E are the common denominator in this disorder, a number of apparent paradoxes concerning its pathogenesis still exist. However, studies in transgenic animals a...

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Type III hyperlipoproteinemia exaggerated by Sheehan's syndrome with advanced systemic atherosclerosis: a 28-year clinical course.

A 38-year-old Japanese woman was admitted to hospital for further examination of systemic xanthomas. She had a past history of genital bleeding during her third delivery at the age of 21 years. She was diagnosed with Sheehan's syndrome. Her serum total cholesterol and triglyceride concentrations were 500 and 898 mg/dl, respectively. She was also diagnosed as having type III hyperlipoproteinemia...

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Gestational hyperlipidemic pancreatitis caused by type III hyperlipoproteinemia with apolipoprotein E2/E2 homozygote.

To the Editor: Gestational hyperlipidemic pancreatitis is an uncommon but serious disorder. In women having type I, IV, or V hyperlipoproteinemia (HLP), the superimposition of the physiologic hyperlipidemia of pregnancy may lead to acute pancreatitis. Here, we present a case of gestational hyperlipidemic pancreatitis associated with apo E2/E2 homozygous type III HLP. A 39-year-old gravida 3, pa...

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Biophysical Analysis of Apolipoprotein E3 Variants Linked with Development of Type III Hyperlipoproteinemia

BACKGROUND Apolipoprotein E (apoE) is a major protein of the lipoprotein transport system that plays important roles in lipid homeostasis and protection from atherosclerosis. ApoE is characterized by structural plasticity and thermodynamic instability and can undergo significant structural rearrangements as part of its biological function. Mutations in the 136-150 region of the N-terminal domai...

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Expression of type III hyperlipoproteinemia in apolipoprotein E2 (Arg158 --> Cys) homozygotes is associated with hyperinsulinemia.

Type III hyperlipoproteinemia (HLP) is mainly found in homozygous carriers of apolipoprotein E2 (apoE2, Arg158-->Cys). Only a small percentage (< 5%) of these apoE2 homozygotes develops hyperlipidemia, indicating that additional environmental and genetic factors contribute to the expression of type III HLP. In the present study, first, the prevalence of type III HLP among apoE2 homozygotes was ...

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ژورنال

عنوان ژورنال: New England Journal of Medicine

سال: 1982

ISSN: 0028-4793,1533-4406

DOI: 10.1056/nejm198207083070202