Myelomatosis with type III hyperlipoproteinemia - clinical and metabolic studies

Cortese, Claudio, Lewis, Barry, Miller, Norman E. , Peyman, Michael A. , Rao, Suresh N. , Slavin, Brenda, Sule, Usha, Turner, Peter R. , Utermann, Gerd, Wing, Anthony J. , Weight, Michael and Wootton, Richard (1982) Myelomatosis with type III hyperlipoproteinemia - clinical and metabolic studies. New England Journal of Medicine, 307 2: 79-83. doi:10.1056/NEJM198207083070202

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Author Cortese, Claudio
Lewis, Barry
Miller, Norman E.
Peyman, Michael A.
Rao, Suresh N.
Slavin, Brenda
Sule, Usha
Turner, Peter R.
Utermann, Gerd
Wing, Anthony J.
Weight, Michael
Wootton, Richard
Title Myelomatosis with type III hyperlipoproteinemia - clinical and metabolic studies
Journal name New England Journal of Medicine   Check publisher's open access policy
ISSN 0028-4793
1533-4406
Publication date 1982-07-01
Sub-type Article (original research)
DOI 10.1056/NEJM198207083070202
Open Access Status File (Publisher version)
Volume 307
Issue 2
Start page 79
End page 83
Total pages 5
Place of publication Waltham, MA, United States
Publisher Massachusetts Medical Society
Language eng
Abstract We investigated the metabolism of intermediate-density lipoproteins (IDL [1.006 to 1.019 g per milliliter]) and low-density lipoproteins (LDL [1.019 to 1.063 g per milliliter]) in two men with Type III hyperlipoproteinemia associated with myelomatosis. In vivo kinetic studies using Radio-labeled autologous lipoproteins demonstrated a greatly reduced fractional catabolic rate of IDL, relative to control values (patients vs. normal, 0.006 and 0.025 per hour vs. 0.20±0.08 per hour [mean ±S.E.M.]) and a greatly prolonged IDL-to-LDL conversion time (45 and 17 hours vs. 5.4±1.6 hours). In studies in vitro, LDL from both patients failed to bind to the LDL receptor of normal blood lymphocytes, whereas LDL from subjects with familial Type III hyperlipoproteinemia bound normally to the receptor. In one patient immunoglobulin was shown to be associated with IDL and LDL. Thus, hyperlipoproteinemia reflected an impaired metabolism of IDL, probably secondary to the binding of immunoglobulin to the lipoproteins. A similar impairment of receptor-mediated LDL catabolism did not elevate the plasma LDL concentration because of the low IDL-to-LDL conversion rate.
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Medicine Publications
 
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