Skip to main content
NCBI home page
The Journal of Clinical Investigation logoLink to The Journal of Clinical Investigation
. 1971 May;50(5):1131–1140. doi: 10.1172/JCI106585

Plasma lipoproteins in familial lecithin: cholesterol acyltransferase deficiency: physical and chemical studies of low and high density lipoproteins

Kaare R Norum 1,2, John A Glomset 1,2, Alex V Nichols 1,2, Trudy Forte 1,2
PMCID: PMC292036  PMID: 5552410

Abstract

Low density lipoproteins (LDL) and high density lipoproteins (HDL) from the plasma of patients with familial lecithin: cholesterol acyltransferase (LCAT) deficiency have been characterized by gel filtration, analytical ultracentrifugation, and gel electrophoresis, and their relative content of lipid and protein has been determined. The LDL of d 1.019-1.063 g/ml show marked heterogeneity. A subfraction of the LDL emerges from columns of 2% agarose gel with the void volume, has corrected flotation rates (Sf°) in the range of 20-400, and contains 4-10 times as much unesterified cholesterol, phosphatidylcholine, and triglyceride per mg protein as normal LDL. A major subfraction of the LDL emerges from the gel in the same general position as normal LDL, but exhibits somewhat higher flotation rates and contains 1.5-3 times as much unesterified cholesterol and phosphatidylcholine and 13 times as much triglyceride per mg protein. The HDL, shown to be heterogeneous in earlier studies, are mainly comprised of molecules which have flotation rates of F1.20 3-20, migrate in the α1-α2 region on electrophoresis, and contain about 12 times as much unesterified cholesterol and 5 times as much phosphatidylcholine per mg protein as normal HDL. Smaller molecules are also detected, which have flotation rates of F1.20 0-3, migrate in the prealbumin region on electrophoresis, and contain only slightly more unesterified cholesterol and phosphatidylcholine per mg protein than normal HDL.

Full text

PDF
1131

Images in this article

1133Image
on p.1133
1137Image
on p.1137

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. AVIGAN J. A method for incorporating cholesterol and other lipides into serum lipoproteins in vitro. J Biol Chem. 1959 Apr;234(4):787–790. [PubMed] [Google Scholar]
  2. DIETRICH F. Zur Differenzierung der Lipoproteide des Serums mittels präparativer Elektrophoresemethoden. Hoppe Seylers Z Physiol Chem. 1955 Dec 22;302(4-6):227–235. [PubMed] [Google Scholar]
  3. DOBBIN V., GOFMAN J. W., NICHOLS A. V. Influence of dietary factors upon human serum lipoprotein concentrations. Geriatrics. 1957 Jan;12(1):7–17. [PubMed] [Google Scholar]
  4. Forte T., Norum K. R., Glomset J. A., Nichols A. V. Plasma lipoproteins in familial lecithin: cholesterol acyltransferase deficiency: structure of low and high density lipoproteins as revealed by elctron microscopy. J Clin Invest. 1971 May;50(5):1141–1148. doi: 10.1172/JCI106586. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. GUSTAFSON A., ALAUPOVIC P., FURMAN R. H. STUDIES OF THE COMPOSITION AND STRUCTURE OF SERUM LIPOPROTEINS: ISOLATION, PURIFICATION, AND CHARACTERIZATION OF VERY LOW DENSITY LIPOPROTEINS OF HUMAN SERUM. Biochemistry. 1965 Mar;4:596–605. doi: 10.1021/bi00879a033. [DOI] [PubMed] [Google Scholar]
  6. Gjone E., Norum K. R. Familial serum cholesterol ester deficiency. Clinical study of a patient with a new syndrome. Acta Med Scand. 1968 Jan-Feb;183(1-2):107–112. [PubMed] [Google Scholar]
  7. Gjone E., Norum K. R. Plasma lecithin-cholesterol acyltransferase and erythrocyte lipids in liver disease. Acta Med Scand. 1970 Mar 3;187(3):153–161. doi: 10.1111/j.0954-6820.1970.tb02925.x. [DOI] [PubMed] [Google Scholar]
  8. Glomset J. A., Norum K. R., King W. Plasma lipoproteins in familial lecithin: cholesterol acyltransferase deficiency: lipid composition and reactivity in vitro. J Clin Invest. 1970 Oct;49(10):1827–1837. doi: 10.1172/JCI106400. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Hamnström B., Gjone E., Norum K. R. Familial plasma lecithin: cholesterol acyltransferase deficiency. Br Med J. 1969 May 3;2(5652):283–286. doi: 10.1136/bmj.2.5652.283. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. LEES R. S., HATCH F. T. Sharper separation of lipoprotein species by paper electrophoresis in albumin-containing buffer. J Lab Clin Med. 1963 Mar;61:518–528. [PubMed] [Google Scholar]
  11. Lees R. S. Immunoassay of plasma low-density lipoproteins. Science. 1970 Jul 31;169(3944):493–495. doi: 10.1126/science.169.3944.493. [DOI] [PubMed] [Google Scholar]
  12. Nichols A. V. Human serum lipoproteins and their interrelationships. Adv Biol Med Phys. 1967;11:109–158. doi: 10.1016/b978-1-4832-3107-5.50008-x. [DOI] [PubMed] [Google Scholar]
  13. Noble R. P. Electrophoretic separation of plasma lipoproteins in agarose gel. J Lipid Res. 1968 Nov;9(6):693–700. [PubMed] [Google Scholar]
  14. Norum K. R., Borsting S., Grundt I. Familial lecithin: cholesterol acyltransferase deficiency. Study of two new patients and their close relatives. Acta Med Scand. 1970 Oct;188(4):323–326. [PubMed] [Google Scholar]
  15. RUSS E. M., RAYMUNT J., BARR D. P. Lipoproteins in primary biliary cirrhosis. J Clin Invest. 1956 Feb;35(2):133–144. doi: 10.1172/JCI103257. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. SCHONENBERGER M., SCHULTZE H. E., SCHWICK G. Uber ein Präalbumin des menschlichen Serums. Biochem Z. 1956;328(4):267–284. [PubMed] [Google Scholar]
  17. SMITH E. L., BROWN D. M., WEIMER H. E., WINZLER R. J. Sedimentation, diffusion, and molecular weight of a mucoprotein from human plasma. J Biol Chem. 1950 Aug;185(2):569–575. [PubMed] [Google Scholar]
  18. Seidel D., Alaupovic P., Furman R. H. A lipoprotein characterizing obstructive jaundice. I. Method for quantitative separation and identification of lipoproteins in jaundiced subjects. J Clin Invest. 1969 Jul;48(7):1211–1223. doi: 10.1172/JCI106085. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Switzer S. Plasma lipoproteins in liver disease. I. Immunologically distinct low-density lipoproteins in patients with biliary obstruction. J Clin Invest. 1967 Nov;46(11):1855–1866. doi: 10.1172/JCI105676. [DOI] [PMC free article] [PubMed] [Google Scholar]

Articles from Journal of Clinical Investigation are provided here courtesy of American Society for Clinical Investigation

RESOURCES