High receptor binding affinity of lipoproteins in atypical dysbetalipoproteinemia (type III hyperlipoproteinemia).
Open accessAbstract
Familial dysbetalipoproteinemia (or type III hyperlipoproteinemia) is characterized by the presence of abnormal, cholesteryl ester-rich beta-very low density lipoproteins (beta-VLDL) in the plasma. Subjects with typical dysbetalipoproteinemia are homozygous for an amino acid substitution in apolipoprotein (apo-) E at residue 158 and have defective apo-E-mediated binding of both pre-beta-VLDL and beta-VLDL to apo-B,E(LDL) (or LDL) receptors (1988. Chappell, D.A., J. Clin. Invest. 82:628-639). To understand the effect of substitutions in apo-E at sites other than residue 158, nine dysbetalipoproteinemic (dys-beta) subjects who were either homozygous or heterozygous for substitutions in apo-E at atypical sites were studied. These substitutions occurred at residue 142 (n = 6), 145 (n = 2), or 146 (n = 1) and are known to cause less defective binding than does the 158 substitution. The chemical composition and electrophoretic mobility of pre-beta-VLDL and beta-VLDL from atypical and typical dys-beta subjects were indistinguishable. However, lipoproteins from atypical and typical dys-beta subjects differed in their affinity for the apo-B,E(LDL) receptor on cultured human fibroblasts. The pre-beta-VLDL and beta-VLDL from atypical dys-beta subjects had 640- or 17-fold higher affinity, respectively, than did corresponding lipoproteins from typical dys-beta subjects. The higher binding affinity of lipoproteins from atypical dys-beta subjects was associated with a higher ratio of apo-E to total apo-C. Since higher binding affinity should cause more rapid receptor-mediated clearance of beta-VLDL in atypical than in typical dys-beta subjects in vivo, the mechanism of beta-VLDL accumulation may differ in these two groups.
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LDL-receptor binding defects for these apoE variants do not correlate well with the severity of dyslipidemia, indicating that the variants may carry additional properties contributing to pathogenic potential.
“However, the LDL-receptor binding defects for these apoE variants do not correlate well with the severity of dyslipidemia, indicating that these variants may carry additional properties that contribute to their pathogenic potential.”
From Biophysical Analysis of Apolipoprotein E3 Variants Linked with Development of Type III Hyperlipoproteinemia (2011) · cites this paper as [38]
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Georgiadou, D., Chroni, A., Vezeridis, A., Zannis, V. I., & Stratikos, E. (2011) · PLoS ONE · doi:10.1371/journal.pone.0027037 · Cited by 24 · see citing works
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Fotakis, P., Vezeridis, A., Dafnis, I., Chroni, A., Kardassis, D., & Zannis, V. I. (2014) · Journal of Lipid Research · doi:10.1194/jlr.m048348 · Cited by 4 · see citing works
- Authors
- Chappell, D. A.
- Affiliations
- University of California, San Francisco (US)
- Corresponding author
- Chappell, D A
- Venue
- Journal of Clinical Investigation
- Published
- 1989-12-01
- Volume
- 84
- Issue
- 6
- Pages
- 1906-1915
- ISSN
- 0021-9738 (ISSN-L)
- PMC
- PMC304071
- OpenAlex
- W2076742691
- Cited by
- 22
- References
- 3
- Citations by year
- 2022: 1 · 2021: 1 · 2014: 2
- FWCI
- 1.55
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Journal of Clinical Investigation (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (02024)
- University of California, San Francisco
- National Heart, Lung, and Blood Institute (HL-02024, HL 07216, HL-14230)
- 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, familial dysbetalipoproteinemia, apolipoprotein E, beta-VLDL, LDL receptor, receptor binding affinity, receptor-mediated clearance, apolipoprotein C, fibroblasts
- MeSH terms
- Adolescent, Adult, Aged, Animals, Apolipoproteins B, Apolipoproteins E, Binding, Competitive, Cells, Cultured, Child, Cholesterol, Dietary, Dogs, Electrophoresis, Polyacrylamide Gel, Female, Fibroblasts, Humans, Hyperlipoproteinemia Type III, Lipoproteins, LDL, Lipoproteins, VLDL, Male, Middle Aged, Mutation, Rabbits, Receptors, Cell Surface, Receptors, LDL, Receptors, Lipoprotein
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