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Related Experiment Videos

Plasma platelet activating factor-acetylhydrolase (PAF-AH).

Ken Karasawa1, Ayako Harada, Noriko Satoh

  • 1Faculty of Pharmaceutical Sciences, Teikyo University, Sagamiko, Kanagawa, Japan. karasawa@pharm.teikyo-u.ac.jp

Progress in Lipid Research
|January 28, 2003
PubMed
Summary
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Platelet-activating factor-acetylhydrolase (PAF-AH) deficiency, common in Japanese populations due to the V279F mutation, is linked to inflammatory diseases. PAF-AH substitution shows promise for treating these conditions.

Area of Science:

  • Biochemistry
  • Enzymology
  • Genetics

Background:

  • Platelet-activating factor-acetylhydrolase (PAF-AH) comprises intracellular (Ib, II) and plasma isoforms with distinct biochemical properties.
  • Plasma PAF-AH and intracellular isoform II degrade PAF and oxidized phospholipids, influencing inflammatory processes.
  • Altered plasma PAF-AH activity is observed in inflammatory diseases like asthma, sepsis, and vascular conditions.

Purpose of the Study:

  • To investigate the role of plasma PAF-AH in inflammatory diseases.
  • To explore the implications of genetic variations in plasma PAF-AH, particularly the V279F mutation.
  • To evaluate the therapeutic potential of PAF-AH substitution in inflammatory disease models.

Main Methods:

  • Analysis of plasma PAF-AH activity in patients with inflammatory diseases.

Related Experiment Videos

  • Identification and characterization of plasma PAF-AH genetic polymorphisms, including V279F.
  • Assessment of therapeutic efficacy using recombinant plasma PAF-AH and gene transfer in animal models.

    • Main Results:

    • A common loss-of-function mutation (V279F) in plasma PAF-AH affects over 30% of Japanese subjects, identified as a potential genetic risk factor.
    • Enzymatic deficiency due to this polymorphism is linked to various inflammatory diseases.
    • Administration of recombinant plasma PAF-AH or gene transfer ameliorated disease pathology in animal studies.

    Conclusions:

    • Plasma PAF-AH deficiency, particularly the V279F polymorphism, represents a significant genetic risk factor for inflammatory diseases in Japanese populations.
    • PAF-AH substitution therapy offers a novel and effective approach for treating inflammatory conditions.
    • Detecting plasma PAF-AH gene polymorphisms and enzyme activity abnormalities can aid in diagnosing inflammatory diseases.