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Roles of LonP1 in Oral-Maxillofacial Developmental Defects and Tumors: A Novel Insight

Journal

Publisher

MDPI
DOI: 10.3390/ijms232113370

Keywords

LonP1; mitochondrial Lon protease; proteolysis; molecular chaperone; mitochondrial DNA; oral and maxillofacial oncology; CODAS

Funding

  1. National Natural Science Foundation of China [81371107, 81870737, 81771098]
  2. Natural Science Foundation of Guangdong Province [2021A1515011779]
  3. Guangdong Financial Fund for High-Caliber Hospital Construction [174-2018XMZC-0001-03-0125/D-02]

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LonP1 plays a central role in mitochondrial function and energy metabolism, and has been associated with tumors and developmental disorders; High expression of LonP1 in oral and maxillofacial tumors makes it a potential anticancer therapy target; Further research on LonP1-related diseases is crucial for understanding LonP1-associated oral phenotypes and their molecular mechanisms.
Recent studies have indicated a central role for LonP1 in mitochondrial function. Its physiological functions include proteolysis, acting as a molecular chaperone, binding mitochondrial DNA, and being involved in cellular respiration, cellular metabolism, and oxidative stress. Given its vital role in energy metabolism, LonP1 has been suggested to be associated with multi-system neoplasms and developmental disorders. In this study, we investigated the roles, possible mechanisms of action, and therapeutic roles of LonP1 in oral and maxillofacial tumor development. LonP1 was highly expressed in oral-maxillofacial cancers and regulated their development through a sig-naling network. LonP1 may therefore be a promising anticancer therapy target. Mutations in LONP1 have been found to be involved in the etiology of cerebral, ocular, dental, auricular, and skeletal syndrome (CODAS). Only patients carrying specific LONP1 mutations have certain dental abnormalities (delayed eruption and abnormal morphology). LonP1 is therefore a novel factor in the development of oral and maxillofacial tumors. Greater research should therefore be conducted on the diagnosis and therapy of LonP1-related diseases to further define LonP1-associated oral phenotypes and their underlying molecular mechanisms.

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