Type III Hyperlipoproteinemia: Diagnosis, Molecular Defects, Pathology, and Treatment
Abstract
Type III hyperlipoproteinemia is characterized by increased plasma levels of triglycerides and cholesterol, palmar-tuberoeruptive xanthoma, and premature cardiovascular disease. Three major classes of molecular defects will predispose patients to develop type III hyperlipoproteinemia: a deficiency in apolipoprotein E, a structural defect in the E apolipoprotein, and a functional defect in the liver receptor system. Most patients with type III hyperlipoproteinemia have a structural defect in apolipoprotein E associated with increased synthesis and decreased catabolism of apolipoprotein E, delayed catabolism of chylomicron remnants, and development of plasma lipoprotein abnormalities characteristic of type III hyperlipoproteinemia. Analysis of cardiovascular disease in patients with type III hyperlipoproteinemia showed extensive coronary and peripheral vascular atherosclerosis indistinguishable from the atherosclerosis of non-hyperlipidemic and other dyslipoproteinemic patients. The xanthoma and elevated plasma cholesterol and triglyceride levels in patients with type III hyperlipoproteinemia respond to dietary and drug therapy.
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APOE ε22 homozygotes have a propensity to develop type III hyperlipoproteinemia, which can be triggered by secondary factors such as age, diabetes, hypothyroidism, and obesity.
“APOE ε22 homozygotes have the propensity to develop type III hyperlipoproteinemia, which can be triggered by secondary factors such as age, diabetes, hypothyroidism and obesity [9,52].”
From Plasma levels of apolipoprotein E, APOE genotype and risk of dementia and ischemic heart disease: A review (2016) · cites this paper as [52]
Type III hyperlipoproteinemia is characterized by elevations of both plasma cholesterol and triglycerides due to an increase in chylomicron remnants, VLDL, and IDL.
“Type III hyperlipoproteinemia is characterized by elevations of both plasma cholesterol and triglycerides due to an increase in chylomicron remnants, VLDL and IDL [9,26,52].”
From Plasma levels of apolipoprotein E, APOE genotype and risk of dementia and ischemic heart disease: A review (2016) · cites this paper as [52]
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- Authors
- BREWER, H. B., ZECH, L. A., GREGG, R. E., SCHWARTZ, D., & SCHAEFER, E. J.
- Affiliations
- Bethesda, Maryland
- Corresponding author
- BREWER, H. BRYAN
- ORCID
- SCHAEFER, ERNST J.
- Venue
- Annals of Internal Medicine
- Publisher
- American College of Physicians
- Published
- 1983-05-01
- Volume
- 98
- Issue
- 5_Part_1
- Pages
- 623-640
- ISSN
- 0003-4819 (ISSN-L)
- 1539-3704
- OpenAlex
- W2098945650
- Cited by
- 226
- References
- 2
- Citations by year
- 2026: 1 · 2024: 3 · 2023: 2 · 2022: 2 · 2021: 1 · 2020: 2 · 2018: 1 · 2017: 3 · 2016: 2 · 2015: 2 · 2014: 1 · 2013: 1 · 2012: 5
- FWCI
- 12.04
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Annals of Internal Medicine (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Lipid metabolism and disorders
- Health Sciences › Medicine › Surgery › Lipoproteins and Cardiovascular Health
- Health Sciences › Medicine › Surgery › Cholesterol and Lipid Metabolism
- Keywords
- type III hyperlipoproteinemia, apolipoprotein E deficiency, chylomicron remnants, xanthoma, atherosclerosis, cardiovascular disease, hypertriglyceridemia, hypercholesterolemia, dietary therapy, pharmacotherapy
- MeSH terms
- Adolescent, Adult, Aged, Apolipoproteins, Clofibrate, Coronary Vessels, Diet, Female, Humans, Hyperlipoproteinemia Type III, Intestinal Mucosa, Lipids, Lipoproteins, Liver, Male, Middle Aged, Molecular Weight, Phenotype, Xanthomatosis
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