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CLPP gene

caseinolytic mitochondrial matrix peptidase proteolytic subunit

Normal Function

The CLPP gene provides instructions for making the ClpP subunit protein. Multiple copies of this protein interact with each other to form a barrel-shaped chamber known as the ClpP complex. This complex is one of two parts of the ClpXP protease, which breaks down abnormally folded proteins. The other part of the ClpXP protease, called the ClpX complex, unfolds the abnormal proteins and feeds them into the chamber formed by the ClpP complex, where they are broken down into small fragments.

The ClpP complex is found in structures in the cell called mitochondria, which are the energy-producing centers of cells.

Health Conditions Related to Genetic Changes

Perrault syndrome

At least three mutations in the CLPP gene have been found in families with Perrault syndrome, a condition characterized by hearing loss in affected males and females and abnormalities of the ovaries in affected females. The CLPP gene mutations involved in Perrault syndrome likely alter the structure of the barrel-shaped chamber formed by the ClpP complex. These changes may impair the breakdown of misfolded mitochondrial proteins, which could impact mitochondrial function; however, it is unclear how these changes lead to the features of Perrault syndrome.

More About This Health Condition

Other Names for This Gene

  • ATP-dependent Clp protease proteolytic subunit, mitochondrial
  • ATP-dependent protease ClpAP, proteolytic subunit, human
  • ClpP caseinolytic peptidase ATP-dependent, proteolytic subunit
  • ClpP caseinolytic protease, ATP-dependent, proteolytic subunit homolog
  • endopeptidase Clp
  • PRLTS3
  • putative ATP-dependent Clp protease proteolytic subunit, mitochondrial

Additional Information & Resources

Tests Listed in the Genetic Testing Registry

Scientific Articles on PubMed

Gene and Variant Databases


  • Baker TA, Sauer RT. ClpXP, an ATP-powered unfolding and protein-degradation machine. Biochim Biophys Acta. 2012 Jan;1823(1):15-28. doi: 10.1016/j.bbamcr.2011.06.007. Epub 2011 Jun 27. Citation on PubMed or Free article on PubMed Central
  • Jenkinson EM, Rehman AU, Walsh T, Clayton-Smith J, Lee K, Morell RJ, Drummond MC, Khan SN, Naeem MA, Rauf B, Billington N, Schultz JM, Urquhart JE, Lee MK, Berry A, Hanley NA, Mehta S, Cilliers D, Clayton PE, Kingston H, Smith MJ, Warner TT; University of Washington Center for Mendelian Genomics; Black GC, Trump D, Davis JR, Ahmad W, Leal SM, Riazuddin S, King MC, Friedman TB, Newman WG. Perrault syndrome is caused by recessive mutations in CLPP, encoding a mitochondrial ATP-dependent chambered protease. Am J Hum Genet. 2013 Apr 4;92(4):605-13. doi: 10.1016/j.ajhg.2013.02.013. Epub 2013 Mar 28. Citation on PubMed or Free article on PubMed Central
  • Kang SG, Dimitrova MN, Ortega J, Ginsburg A, Maurizi MR. Human mitochondrial ClpP is a stable heptamer that assembles into a tetradecamer in the presence of ClpX. J Biol Chem. 2005 Oct 21;280(42):35424-32. doi: 10.1074/jbc.M507240200. Epub 2005 Aug 22. Citation on PubMed
  • Yu AY, Houry WA. ClpP: a distinctive family of cylindrical energy-dependent serine proteases. FEBS Lett. 2007 Jul 31;581(19):3749-57. doi: 10.1016/j.febslet.2007.04.076. Epub 2007 May 8. Citation on PubMed

The information on this site should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.