Type III Hyperlipoproteinemia: Still Worth Considering?
Abstract
Familial type III hyperlipoproteinemia (HLP) was first recognized as a distinct entity over 60 years ago. Since then, it has proven to be instructive in identifying the key role of apolipoprotein E (apoE) in removal of the remnants of very low density lipoproteins and chylomicrons produced by the action of lipoprotein lipase on these triglyceride-transporting lipoproteins. It has additionally shed light on the potent atherogenicity of the remnant lipoproteins. This review describes the history of development of our understanding of type III HLP, discusses the several genetic variants of apoE that play roles in the genesis of type III HLP, and describes the remarkable responsiveness of this fascinating disorder to lifestyle modification, especially carbohydrate restriction and calorie restriction, and, when required, the addition of pharmacotherapy.
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More than 90% of affected individuals with familial type III HLP are APOE2 homozygotes, with the remainder having rare APOE variants, including those causing apoE deficiency or compound heterozygotes of APOE variants and APOE2.
“In fact, more than 90% of affected individuals are APOE2 homozygotes, with the remainder having rare APOE variants, including those causing apoE deficiency or compound heterozygotes of APOE variants and APOE2 23, 25-28).”
From Impact of Apolipoprotein E Variants: A Review of Naturally Occurring Variants and Clinical Features (2025) · cites this paper as [25]
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Effects of <i>APOC3</i> Heterozygous Deficiency on Plasma Lipid and Lipoprotein Metabolism
Reyes-Soffer, G., Sztalryd, C., Horenstein, R. B., Holleran, S., Matveyenko, A., Thomas, T., Nandakumar, R., Ngai, C., Karmally, W., Ginsberg, H. N., et al. (2019) · Arteriosclerosis, Thrombosis, and Vascular Biology · doi:10.1161/atvbaha.118.311476 · Cited by 97 · see citing works
Impact of Apolipoprotein E Variants: A Review of Naturally Occurring Variants and Clinical Features
Matsunaga, A., & Saito, T. (2025) · Journal of Atherosclerosis and Thrombosis · doi:10.5551/jat.65393 · Cited by 14 · see citing works
- Authors
- Blum, C. B.
- Affiliations
- Columbia University Irving Medical Center (US)
- Corresponding author
- Blum, Conrad B.
- Venue
- Progress in Cardiovascular Diseases
- Publisher
- Elsevier BV
- Published
- 2016-09
- Volume
- 59
- Issue
- 2
- Pages
- 119-124
- ISSN
- 0033-0620 (ISSN-L)
- OpenAlex
- W2510651723
- Cited by
- 39
- References
- 6
- Citations by year
- 2026: 1 · 2025: 4 · 2024: 3 · 2023: 4 · 2022: 4 · 2021: 6 · 2020: 3 · 2019: 3 · 2018: 10 · 2017: 1
- FWCI
- 0.81
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Progress in Cardiovascular Diseases (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 › Endocrinology, Diabetes and Metabolism › Diabetes, Cardiovascular Risks, and Lipoproteins
- Keywords
- type III hyperlipoproteinemia, familial hypertriglyceridemia, apolipoprotein E, remnant lipoproteins, VLDL, chylomicrons, lipoprotein lipase, atherogenicity, carbohydrate restriction, caloric restriction, lifestyle modification, pharmacotherapy
- MeSH terms
- Hypolipidemic Agents, Apolipoproteins E, Humans, Hyperlipoproteinemia Type III, Disease Management, Risk Reduction Behavior
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