Saccharopine, a lysine degradation intermediate, is a mitochondrial toxin

  • J Cell Biol. 2019 Feb 4;218(2):391-392. doi: 10.1083/jcb.201901033.
João Leandro  1 ,  Sander M Houten  2
Affiliations
  • 1. Department of Genetics and Genomic Sciences and Icahn Institute for Data Science and Genomic Technology, Icahn School of Medicine at Mount Sinai, New York, NY.
  • 2. Department of Genetics and Genomic Sciences and Icahn Institute for Data Science and Genomic Technology, Icahn School of Medicine at Mount Sinai, New York, NY [email protected].
PMID: 30651301 DOI: 10.1083/jcb.201901033
Abstract

Saccharopine, a nonproteinogenic amino acid originally isolated from the yeast Saccharomyces cerevisiae, is an intermediate in lysine metabolism. In this issue, Zhou et al. (2019. J. Cell Biol. https://doi.org./10.1083/jcb.201807204) show that abnormal accumulation of saccharopine results in defective mitochondrial dynamics and function in worm and mouse models.

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