Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors.

@article{Gispert2013LossOM,
  title={Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors.},
  author={Suzana Gispert and Dajana Parganlija and Michael Klinkenberg and Stefan Dr{\"o}se and Ilka Wittig and Michel Mittelbronn and Pawel Grzmil and Sebastian Koob and Andrea Hamann and Michael A Walter and Finja B{\"u}chel and Thure Adler and Martin Hrabě de Angelis and Dirk H Busch and Andreas Zell and Andreas S. Reichert and Ulrich Brandt and Heinz D. Osiewacz and Marina Jendrach and Georg Auburger},
  journal={Human molecular genetics},
  year={2013},
  volume={22 24},
  pages={4871-87}
}
The caseinolytic peptidase P (CLPP) is conserved from bacteria to humans. In the mitochondrial matrix, it multimerizes and forms a macromolecular proteasome-like cylinder together with the chaperone CLPX. In spite of a known relevance for the mitochondrial unfolded protein response, its substrates and tissue-specific roles are unclear in mammals. Recessive CLPP mutations were recently observed in the human Perrault variant of ovarian failure and sensorineural hearing loss. Here, a first… CONTINUE READING
Recent Discussions
This paper has been referenced on Twitter 2 times over the past 90 days. VIEW TWEETS
33 Citations
118 References
Similar Papers

Citations

Publications citing this paper.
Showing 1-10 of 33 extracted citations

References

Publications referenced by this paper.
Showing 1-10 of 118 references

A mutation in PNPT1, encoding mitochondrial-RNA-import protein PNPase, causes hereditary hearing loss

  • S. von Ameln, G. Wang, +8 authors A.E
  • Am. J. Hum. Genet.,
  • 2012

Similar Papers

Loading similar papers…