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Phytol is lethal for Amacr-deficient mice.

Eija M Selkälä1, Remya R Nair1, Werner Schmitz2

  • 1Faculty of Biochemistry and Molecular Medicine, University of Oulu, P.O. Box 5400, FI-90014, Finland; Biocenter Oulu, University of Oulu, Finland.

Biochimica Et Biophysica Acta
|August 7, 2015
PubMed
Summary

α-Methylacyl-CoA racemase (Amacr) deficiency causes severe liver failure in mice fed phytol. Amacr is crucial for detoxifying α-methyl-branched fatty acids, preventing phytol-induced toxicity.

Keywords:
Alpha-methylacyl-CoA racemaseKnock-out mouse modelPeroxisomePhytolRefsum disease

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Area of Science:

  • Biochemistry
  • Metabolic Disorders
  • Toxicology

Background:

  • α-Methylacyl-CoA racemase (Amacr) is essential for bile acid synthesis and fatty acid metabolism.
  • Amacr deficiency leads to accumulation of toxic metabolites like pristanic and phytanic acids, causing neurological symptoms in humans.
  • Amacr-deficient mice are asymptomatic on standard diets but susceptible to phytol-induced disease.

Purpose of the Study:

  • To investigate the mechanisms of phytol-induced disease in Amacr-deficient mice.
  • To elucidate the role of Amacr in the detoxification of phytol metabolites.
  • To understand the molecular response to phytol in the liver.

Main Methods:

  • Feeding Amacr-deficient (Amacr-/-) and wild-type mice with a phytol-enriched diet.
  • Monitoring survival rates and clinical signs.
  • Performing histological analysis of liver, kidney, and brain tissues.
  • Analyzing serum for liver damage markers.
  • Utilizing microarray analysis to assess gene expression changes in the liver.

Main Results:

  • Amacr-/- mice on a phytol diet exhibited high mortality (all died within 36 weeks) due to liver failure, unlike wild-type mice.
  • Histological examination revealed significant liver inflammation, fibrosis, necrosis, and fatty changes in Amacr-/- mice.
  • Pristanic and phytanic acids accumulated in the livers of Amacr-/- mice fed phytol.
  • Phytol diet induced broad gene expression changes in wild-type mouse livers, indicating complex detoxification pathways.

Conclusions:

  • Amacr is indispensable for detoxifying α-methyl-branched fatty acids derived from phytol.
  • Phytol causes primary liver failure in Amacr-deficient mice, highlighting the enzyme's critical role in preventing phytol toxicity.
  • Amacr deficiency severely impairs the liver's ability to handle phytol metabolites, leading to fatal outcomes.