Mitochondrial Lon protease is a gatekeeper for proteins newly imported into the matrix

Yuichi Matsushima, Kazuya Takahashi, Song Yue, Yuki Fujiyoshi, Hideaki Yoshioka, Masamune Aihara, Daiki Setoyama, Takeshi Uchiumi, Satoshi Fukuchi, Dongchon Kang

Research output: Contribution to journalArticlepeer-review

12 Citations (Scopus)


Human ATP-dependent Lon protease (LONP1) forms homohexameric, ring-shaped complexes. Depletion of LONP1 causes aggregation of a broad range of proteins in the mitochondrial matrix and decreases the levels of their soluble forms. The ATP hydrolysis activity, but not protease activity, of LONP1 is critical for its chaperone-like anti-aggregation activity. LONP1 forms a complex with the import machinery and an incoming protein, and protein aggregation is linked with matrix protein import. LONP1 also contributes to the degradation of imported, aberrant, unprocessed proteins using its protease activity. Taken together, our results show that LONP1 functions as a gatekeeper for specific proteins imported into the mitochondrial matrix.

Original languageEnglish
Article number974
JournalCommunications Biology
Issue number1
Publication statusPublished - Dec 2021

All Science Journal Classification (ASJC) codes

  • Medicine (miscellaneous)
  • General Biochemistry,Genetics and Molecular Biology
  • General Agricultural and Biological Sciences


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