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    Comparison of apolipoprotein B metabolism in familial defective apolipoprotein B and heterogeneous familial hypercholesterolemia.

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    Authors
    Gaffney, Dairena
    Forster, Lorne
    Caslake, Muriel J
    Bedford, Dorothy
    Stewart, J Philip
    Stewart, Grace
    Wieringa, Gilbert E
    Dominiczak, Marek
    Miller, J Paul
    Packard, Chris J
    Affiliation
    Department of Pathological Biochemistry, Glasgow Royal Infirmary, North Glasgow Hospitals University NHS trust, 4th floor, Queen Elizabeth Building, Alexandra Parade, Glasgow G31 2ER, Scotland, UK. dairena.gaffney@clinmed.gla.ac.uk
    Issue Date
    2002-05
    
    Metadata
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    Abstract
    Both defective LDL receptors (familial hypercholesterolaemia, FH) and mutations in apolipoprotein B (apoB) on LDL (familial defective apoB, FDB) give rise to a phenotype of elevated LDL cholesterol. We sought to compare the metabolic basis of the two conditions by examining apoB turnover in FDB and FH subjects. A group comprising three heterozygous and one homozygous FDB subjects were compared with five FH heterozygotes and 17 control subjects using a deuterated leucine tracer. Kinetic parameters were derived by multicompartmental modelling. FH heterozygotes had a reduced delipidation rate for VLDL, which led to a moderate increase in plasma triglyceride. Compared with controls and FH, the FDB subjects converted 44% less IDL to LDL. The LDL FCR was reduced to a similar extent in FDB and FH. In all subjects LDL plasma levels appeared to be regulated by the LDL FCR and the rate of production of small VLDL. We conclude that disturbances in IDL metabolism provide the basis for understanding why FDB is less severe than FH. Our findings suggest that an apoB-LDL receptor interaction is important in the IDL to LDL conversion.
    Citation
    Comparison of apolipoprotein B metabolism in familial defective apolipoprotein B and heterogeneous familial hypercholesterolemia. 2002, 162 (1):33-43 Atherosclerosis
    Journal
    Atherosclerosis
    URI
    http://hdl.handle.net/10541/81173
    PubMed ID
    11947895
    Type
    Article
    Language
    en
    ISSN
    0021-9150
    Collections
    All Christie Publications

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