Defective enzyme protein in lipoprotein lipase deficiency

European Journal of Clinical Investigation
J H AuwerxJ D Brunzell

Abstract

A monoclonal antibody to lipoprotein lipase (LPL) has been used in an enzyme-linked immunosorbent assay (ELISA) for LPL protein mass. Measurement of LPL immunoreactive mass in pre- and postheparin plasma distinguished three classes of abnormalities in patients with classical deficiency of lipoprotein lipase activity. The class I defect consisted of the absence of LPL immunoreactive homodimer in pre- and postheparin plasma compatible with a potential 'null allele'. Patients with a class II defect had almost no LPL immunoreactive mass in preheparin plasma but showed an increase in their LPL mass of 68 +/- 23 ng ml-1 (mean +/- SD) after heparin. Patients with the class III defect had considerable amounts of LPL immunoreactive material in preheparin plasma (159 +/- 190 ng ml-1). Heparin administration, however, caused very little additional release of LPL into the plasma (16 +/- 51 ng ml-1). Thus although both class II and class III patients had an LPL protein with abnormal catalytic activity, class III patients also appeared to have a defect in heparin binding of LPL. To test this hypothesis, postheparin plasma of classes II and III patients was analysed by heparin-Sepharose chromatography. In contrast to class II patients, the LP...Continue Reading

References

Feb 1, 1989·Proceedings of the National Academy of Sciences of the United States of America·S LangloisM R Hayden
May 1, 1989·Arteriosclerosis : an Official Journal of the American Heart Association, Inc·S P BabirakJ D Brunzell
Jan 1, 1986·Cold Spring Harbor Symposia on Quantitative Biology·D W RussellJ L Goldstein
Jan 1, 1986·Advances in Experimental Medicine and Biology·J D BrunzellJ Frolich
Mar 27, 1987·Science·K L WionR M Lawn
Jan 1, 1985·Annual Review of Genetics·J T Stout, C T Caskey

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Citations

Jan 1, 1995·Molecular Aspects of Medicine·M J Halpern
Dec 29, 1999·Clinica Chimica Acta; International Journal of Clinical Chemistry·D A LambertA M Gotto
Oct 1, 1995·Baillière's Clinical Endocrinology and Metabolism·R KirchmairJ R Patsch
May 1, 1990·Proceedings of the National Academy of Sciences of the United States of America·O U BegS S Fojo
Oct 1, 1991·Proceedings of the National Academy of Sciences of the United States of America·U BeisiegelG Bengtsson-Olivecrona
Jun 1, 1992·Journal of Internal Medicine·S S Fojo, H B Brewer
Sep 1, 1991·The Journal of Clinical Investigation·D L SprecherD A Wiginton
Jan 12, 2013·Journal of Lipid Research·Elena Mendoza-BarberáFrancisco Blanco-Vaca
Dec 2, 2011·Journal of Atherosclerosis and Thrombosis·Takanari GotodaUNKNOWN Research Committee for Primary Hyperlipidemia, Research on Measures against Intractable Diseases by the Ministry of Health,
Jan 16, 2007·Clinica Chimica Acta; International Journal of Clinical Chemistry·Junji KobayashiHiroshi Mabuchi
Apr 1, 2014·Biochimica Et Biophysica Acta·André BensadounAnne P Beigneux
Apr 7, 2004·Journal of Atherosclerosis and Thrombosis·Junji Kobayashi
Apr 9, 2015·Frontiers in Physiology·Antonina GiammancoMaurizio R Averna
May 24, 2008·PLoS Computational Biology·Katrin HübnerHermann-Georg Holzhütter
Jan 1, 1992·Critical Reviews in Clinical Laboratory Sciences·J AuwerxK Schoonjans

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