Isoprenoid biosynthesis is not compromised in a Zellweger syndrome mouse model

Isoprenoid biosynthesis is not compromised in a Zellweger syndrome mouse model Because several studies indicated that peroxisomes are important for the biosynthesis of isoprenoids, we wanted to investigate whether a reduced availability of isoprenoids could be one of the pathogenic factors contributing to the severe phenotype of the Pex5 −/− mouse, a model for Zellweger syndrome. Total cholesterol was determined in plasma, brain and liver of newborn mice. In none of these tissues a significant difference was observed between Pex5 −/− and wild type or heterozygous mice. The hepatic ubiquinone content was found to be even higher in Pex5 −/− mice as compared to wild type or heterozygous littermates. To investigate whether the Pex5 −/− fetuses are able to synthesise their own isoprenoids, fibroblasts derived from these mice were incubated with radiolabeled mevalonolactone as a substrate for isoprenoid synthesis. No significant difference was observed between the cholesterol production rates of Pex5 −/− and normal fibroblasts. Our results show that there is no deficiency of isoprenoids in newborn Pex5 −/− mice, excluding the possibility that a lack of these compounds is a determinant factor in the development of the disease state before birth. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids Elsevier

Isoprenoid biosynthesis is not compromised in a Zellweger syndrome mouse model

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Publisher
Elsevier
Copyright
Copyright © 2001 Elsevier Science B.V.
ISSN
1388-1981
DOI
10.1016/S1388-1981(01)00108-1
Publisher site
See Article on Publisher Site

Abstract

Because several studies indicated that peroxisomes are important for the biosynthesis of isoprenoids, we wanted to investigate whether a reduced availability of isoprenoids could be one of the pathogenic factors contributing to the severe phenotype of the Pex5 −/− mouse, a model for Zellweger syndrome. Total cholesterol was determined in plasma, brain and liver of newborn mice. In none of these tissues a significant difference was observed between Pex5 −/− and wild type or heterozygous mice. The hepatic ubiquinone content was found to be even higher in Pex5 −/− mice as compared to wild type or heterozygous littermates. To investigate whether the Pex5 −/− fetuses are able to synthesise their own isoprenoids, fibroblasts derived from these mice were incubated with radiolabeled mevalonolactone as a substrate for isoprenoid synthesis. No significant difference was observed between the cholesterol production rates of Pex5 −/− and normal fibroblasts. Our results show that there is no deficiency of isoprenoids in newborn Pex5 −/− mice, excluding the possibility that a lack of these compounds is a determinant factor in the development of the disease state before birth.

Journal

Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of LipidsElsevier

Published: May 31, 2001

References

  • Cholesterol biosynthesis in dermal fibroblasts from patients with metabolic disorders of peroxisomal origin
    Malle, E.; Oettl, K.; Sattler, W.; Hoefler, G.; Kostner, G.M.
  • A mouse model for Zellweger syndrome
    Baes, M.; Gressens, P.; Baumgart, E.; Carmeliet, P.; Casteels, M.; Fransen, M.; Evrard, P.; Fahimi, D.; Declercq, P.E.; Collen, D.; Van Veldhoven, P.P.; Mannaerts, G.P.
  • Phenotype of patients with peroxisomal disorders subdivided into sixteen complementation groups
    Moser, A.B.; Rasmussen, M.; Naidu, S.; Watkins, P.A.; McGuinness, M.; Hajra, A.K.; Chen, G.; Raymond, G.; Liu, A.; Gordon, D.; Garnaas, K.; Walton, D.S.; Skjeldal, O.H.; Guggenheim, M.A.; Jackson, L.G.; Elias, E.R.; Moser, H.W.

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