Journal of Bioenergetics and Biomembranes

, Volume 50, Issue 6, pp 461–466 | Cite as

Lack of activation of the S113L variant of carnitine palmitoyltransfersase II by cardiolipin

  • Leila Motlagh ScholleEmail author
  • Annemarie Thaele
  • Marie Beckers
  • Beate Meinhardt
  • Stephan Zierz


The phospholipid environment of the mitochondrial inner membrane, which contains large amounts of cardiolipin, could play a key role in transport of the long chain fatty acids. In the present study, the pre-incubation of cardiolipin with the wild type carnitine palmitoyltransferase (CPT) II led to a more than 1.5-fold increase of enzyme activity at physiological temperatures. At higher temperatures, however, there was a pronounced loss of activity. The most frequent variant S113L showed even at 37 °C a great activity loss. Pre-incubation of the wild type with both malonyl-CoA and cardiolipin counteracted the positive effect of cardiolipin. Malonyl-CoA, however, showed no inhibition effect on the variant in presence of cardiolipin. The activity loss in presence of cardiolipin at fever simulating situations was more pronounced for the variant comparing to the wild type. The reason might be a disturbed membrane association or a blockage of the active center of the mutated enzyme.


CPT II deficiency Cardiolipin Membrane association Enzyme activity 



carnitine / acylcarnitine translocase


Coenzyme A


carnitine palmitoyltransferase


5,5΄-dithio-bis-(2-nitrobenzoic acid)


gene coding for human muscle carnitine palmitoyltransferase II


inner mitochondrial membrane


outer mitochondrial membrane


Author contributions

LMS, AT, MB & BM carried out the lab work and data analysis; LMS and SZ designed the study and drafted the manuscript. All authors gave final approval for publication.


This work was supported by the Deutsche Gesellschaft für Muskelkranke (DGM) e.V.


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Leila Motlagh Scholle
    • 1
    Email author
  • Annemarie Thaele
    • 1
  • Marie Beckers
    • 1
  • Beate Meinhardt
    • 1
  • Stephan Zierz
    • 1
  1. 1.Department of NeurologyMartin Luther University Halle-WittenbergHalle (Saale)Germany

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