Metabolism of apolipoproteins B-48 and B-100 of triglyceride-rich lipoproteins in patients with familial dysbetalipoproteinemia.
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The metabolism of apolipoproteins B-48 and B-100 (apo B-48 and B-100) in large triglyceride-rich lipoproteins was studied in three adults with familial dysbetalipoproteinemia (F. dys.) and compared to that of normolipidemic subjects. One Caucasian F. dys. subject was apparently homozygous for the common form of apo E-2, (Arg158----Cys), whereas the two Black subjects were homozygous for a different apo E-2 mutant (Arg145----Cys), which displays much less defective binding to cells than apo E-2 (Arg158----Cys). The lipoproteins were labeled with 125I and injected intravenously into fasted recipients. The results indicate that the terminal catabolism of triglyceride-rich lipoproteins of intestinal and hepatic origin is markedly impaired in apo E2/2 homozygotes with alleles Arg158----Cys and Arg145----Cys; despite long residence times, apo B-48 of chylomicrons and apo B-100 of large very low density lipoproteins are not converted appreciably to intermediate or low density lipoproteins in apo E2/2 homozygotes.
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Patients with dysbetalipoproteinemia have elevations in both cholesterol and triglyceride levels.
“Patients with dysbetalipoproteinemia have elevations in both cholesterol and triglyceride levels (124).”
From Evaluation and Treatment of Hypertriglyceridemia: An Endocrine Society Clinical Practice Guideline (2012) · cites this paper as [124]
The Subjects section and Table I report elevated total cholesterol and triglycerides in two patients with familial dysbetalipoproteinemia. They also state that both levels were normal in the third patient, so the unqualified claim does not hold for every patient studied. (LLM, conf=0.90)
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- Authors
- Stalenhoef, A. F., Malloy, M. J., Kane, J. P., & Havel, R. J.
- Affiliations
- University of California, San Francisco (US)
- ORCID
- Venue
- Journal of Clinical Investigation
- Published
- 1986-09-01
- Volume
- 78
- Issue
- 3
- Pages
- 722-728
- ISSN
- 0021-9738 (ISSN-L)
- PMC
- PMC423660
- OpenAlex
- W2162757044
- Cited by
- 70
- References
- 38
- Citations by year
- 2024: 1 · 2021: 1 · 2016: 2 · 2015: 2 · 2014: 1 · 2013: 3 · 2012: 1
- FWCI
- 7.32
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Journal of Clinical Investigation (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- University of California, San Francisco
- National Heart, Lung, and Blood Institute (HL 14237)
- National Center for Research Resources (RR-79)
- 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
- apolipoprotein B-48, apolipoprotein B-100, triglyceride-rich lipoproteins, familial dysbetalipoproteinemia, apolipoprotein E2, chylomicrons, VLDL, lipoprotein catabolism, lipoprotein metabolism, lipoprotein clearance
- MeSH terms
- Aged, Animals, Apolipoproteins B, Apolipoproteins E, Female, Guinea Pigs, Humans, Hyperlipoproteinemia Type III, Iodine Radioisotopes, Kinetics, Lipoproteins, Male, Middle Aged, Mutation, Triglycerides, Apolipoprotein B-48, Apolipoprotein B-100, Apolipoprotein E2
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