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Hepatic lipase deficiency

P W Connelly1, R A Hegele

  • 1Department of Medicine, St. Michael's Hospital, University of Toronto, Ontario, Canada.

Critical Reviews in Clinical Laboratory Sciences
|January 14, 1999
PubMed
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Hepatic lipase (HL) is crucial for lipoprotein metabolism, influencing HDL cholesterol levels and potentially impacting cardiovascular disease risk. Understanding its physiological role is key to managing lipid disorders.

Area of Science:

  • Biochemistry
  • Lipid Metabolism
  • Enzymology

Background:

  • Hepatic lipase (HL) is an enzyme primarily synthesized by hepatocytes, playing a role in hydrolyzing plasma lipoproteins.
  • It belongs to the lipase superfamily, sharing homology with lipoprotein lipase and pancreatic lipase.
  • HL is involved in converting intermediate-density lipoproteins to LDL and modifying HDL particles.

Purpose of the Study:

  • To elucidate the structure and function of hepatic lipase (HL) in lipoprotein metabolism.
  • To investigate the relationship between HL activity, HDL cholesterol levels, and cardiovascular disease risk.
  • To explore the physiological significance of HL in lipid homeostasis.

Main Methods:

  • Structural analysis of HL, identifying catalytic and lipoprotein-binding sites.

Related Experiment Videos

  • Investigation of N-linked glycosylation sites, specifically Asn-56, for secretion and activity.
  • Correlation analysis of post-heparin HL activity with lipoprotein cholesterol concentrations.
  • Case study of a family with HL deficiency due to specific mutations (Ser267Phe, Thr383Met).

    • Main Results:

    • HL possesses distinct NH2-terminal (catalytic) and COOH-terminal (lipoprotein-binding) domains.
    • Asn-56 glycosylation is essential for HL secretion and enzymatic function.
    • HL activity is inversely correlated with intermediate-density lipoprotein cholesterol in hyperlipidemic subjects.
    • Severe HL deficiency is linked to increased HDL cholesterol, apolipoprotein AI, and HDL triglyceride, and is associated with premature coronary artery disease.

    Conclusions:

    • Hepatic lipase plays a significant role in modifying lipoprotein composition, particularly in HDL metabolism.
    • HL deficiency, especially in conjunction with other hyperlipidemic factors, strongly correlates with premature coronary artery disease.
    • While HL's functions are understood, its precise physiological importance and the implications of its variations require further investigation.