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Isolation of allele-specific, receptor-binding-defective low-density lipoproteins from familial defective apolipoprotein b-100 subjects

Academic Article
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Overview

authors

  • Arnold, K. S.
  • Balestra, M. E.
  • Krauss, R. M.
  • Curtiss, Linda
  • Young, S. G.
  • Innerarity, T. L.

publication date

  • August 1994

journal

  • Journal of Lipid Research  Journal

abstract

  • Familial defective apolipoprotein B-100 (FDB) is a genetic disorder apparently caused by a single amino acid substitution (Arg3500-->Gln) that disrupts the binding of low density lipoproteins (LDL) to the LDL receptor. The plasma of FDB heterozygotes contains a mixture of normal LDL and LDL that is defective in binding to the LDL receptor. In this study, the monoclonal antibody MB19 (which recognizes an immunogenetic polymorphism in apolipoprotein B-100) was used to determine the percentage of defective LDL in the plasma of FDB heterozygotes and to isolate allele-specific receptor-binding-defective LDL. Several FDB heterozygotes were identified who were heterozygous for the MB19 polymorphism: one apolipoprotein B allotype in each of these individuals bound with low affinity to MB19 and possessed the Arg3500-->Gln mutation, whereas the other apolipoprotein B allotype bound with high affinity to MB19 and normally to the LDL receptor. Using MB19 radio-immunoassay, we determined that an average of 73% (range 65-87) of the total LDL from FDB heterozygotes contained the Arg3500-->Gln mutation. Antibody MB19-Sepharose immuno-affinity chromatography was used to separate the receptor-binding-defective LDL from the normal LDL. The isolated LDL contained primarily the Arg3500-->Gln mutation and had only about 9% of normal LDL receptor-binding ability. Finally, the MB19-Sepharose chromatography procedure may be useful for isolating other allele-specific LDL that have functionally significant mutations.

subject areas

  • Alleles
  • Antibodies, Monoclonal
  • Apolipoproteins B
  • Binding, Competitive
  • Cells, Cultured
  • Cholesterol
  • Chromatography, Affinity
  • Fibroblasts
  • Genetic Testing
  • Heterozygote
  • Humans
  • Lipoproteins, LDL
  • Receptors, Lipoprotein
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Research

keywords

  • AFFINITY CHROMATOGRAPHY
  • ALLELES
  • APO-B
  • LDL
  • MONOCLONAL ANTIBODIES
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Identity

International Standard Serial Number (ISSN)

  • 0022-2275

PubMed ID

  • 7989871
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Additional Document Info

start page

  • 1469

end page

  • 1476

volume

  • 35

issue

  • 8

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