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

doi:10.1093/hmg/ddt338

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Article

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

Human molecular genetics - 15 Dec 2013

Gispert Suzana, Parganlija Dajana, Klinkenberg Michael, Dröse Stefan, Wittig Ilka, Mittelbronn Michel, Grzmil Pawel, Koob Sebastian, Hamann Andrea, Walter Michael, Büchel Finja, Adler Thure, Hrabé de Angelis Martin, Busch Dirk H, Zell Andreas, Reichert Andreas S, Brandt Ulrich, Osiewacz Heinz D, Jendrach Marina, Auburger Georg

Abstract excerpt

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...

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Identifiers

PMID: 23851121
DOI: 10.1093/hmg/ddt338
PMCID: PMC7108587

Genes

CLPP
CLPX

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

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Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors.PMID 23851121Read ↗

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