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2011 2
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Page 1
LONP1 targets HMGCS2 to protect mitochondrial function and attenuate chronic kidney disease.
Bai M, Wu M, Jiang M, He J, Deng X, Xu S, Fan J, Miao M, Wang T, Li Y, Yu X, Wang L, Zhang Y, Huang S, Yang L, Jia Z, Zhang A. Bai M, et al. EMBO Mol Med. 2023 Feb 8;15(2):e16581. doi: 10.15252/emmm.202216581. Epub 2023 Jan 11. EMBO Mol Med. 2023. PMID: 36629048 Free PMC article.
LONP1 expression was decreased in human patients and mice with CKD, and tubular-specific Lonp1 overexpression mitigated renal injury and mitochondrial dysfunction in two different models of CKD, but these outcomes were aggravated by Lonp1 deletion. ...Finally …
LONP1 expression was decreased in human patients and mice with CKD, and tubular-specific Lonp1 overexpression mitigated renal …
LonP1 Links Mitochondria-ER Interaction to Regulate Heart Function.
Li Y, Huang D, Jia L, Shangguan F, Gong S, Lan L, Song Z, Xu J, Yan C, Chen T, Tan Y, Liu Y, Huang X, Suzuki CK, Yang Z, Yang G, Lu B. Li Y, et al. Research (Wash D C). 2023 Jun 16;6:0175. doi: 10.34133/research.0175. eCollection 2023. Research (Wash D C). 2023. PMID: 37333972 Free PMC article.
The removal of LonP1 substantially reduces MAM formation and causes mitochondrial fragmentation. ...Consequently, cardiac-specific LonP1 deficiency causes aberrant metabolic reprogramming and pathological heart remodeling. ...
The removal of LonP1 substantially reduces MAM formation and causes mitochondrial fragmentation. ...Consequently, cardiac-specific …
The Role of Lonp1 on Mitochondrial Functions during Cardiovascular and Muscular Diseases.
Zanini G, Selleri V, Malerba M, Solodka K, Sinigaglia G, Nasi M, Mattioli AV, Pinti M. Zanini G, et al. Antioxidants (Basel). 2023 Feb 28;12(3):598. doi: 10.3390/antiox12030598. Antioxidants (Basel). 2023. PMID: 36978846 Free PMC article. Review.
During heart development, Lonp1 allows the metabolic shift from anaerobic glycolysis to mitochondrial oxidative phosphorylation. Knock out of Lonp1 arrests heart development and determines cardiomyocyte apoptosis. ...
During heart development, Lonp1 allows the metabolic shift from anaerobic glycolysis to mitochondrial oxidative phosphorylation. Knoc …
Proteolytic rewiring of mitochondria by LONP1 directs cell identity switching of adipocytes.
Fu T, Sun W, Xue J, Zhou Z, Wang W, Guo Q, Chen X, Zhou D, Xu Z, Liu L, Xiao L, Mao Y, Yang L, Yin Y, Zhang XN, Wan Q, Lu B, Chen Y, Zhu MS, Scherer PE, Fang L, Piao HL, Shao M, Gan Z. Fu T, et al. Nat Cell Biol. 2023 Jun;25(6):848-864. doi: 10.1038/s41556-023-01155-3. Epub 2023 May 22. Nat Cell Biol. 2023. PMID: 37217599
Thermogenic stimulation selectively promotes mitochondrial proteostasis in mature white adipocytes via the mitochondrial protease LONP1. Disruption of LONP1-dependent proteolysis substantially impairs cold- or beta(3) adrenergic agonist-induced white-to-beige identi …
Thermogenic stimulation selectively promotes mitochondrial proteostasis in mature white adipocytes via the mitochondrial protease LONP1 …
Disuse-associated loss of the protease LONP1 in muscle impairs mitochondrial function and causes reduced skeletal muscle mass and strength.
Xu Z, Fu T, Guo Q, Zhou D, Sun W, Zhou Z, Chen X, Zhang J, Liu L, Xiao L, Yin Y, Jia Y, Pang E, Chen Y, Pan X, Fang L, Zhu MS, Fei W, Lu B, Gan Z. Xu Z, et al. Nat Commun. 2022 Feb 16;13(1):894. doi: 10.1038/s41467-022-28557-5. Nat Commun. 2022. PMID: 35173176 Free PMC article.
In humans and mice, disuse-related muscle loss is associated with decreased mitochondrial LONP1 protein. Skeletal muscle-specific ablation of LONP1 in mice resulted in impaired mitochondrial protein turnover, leading to mitochondrial dysfunction. ...Mechanistically, …
In humans and mice, disuse-related muscle loss is associated with decreased mitochondrial LONP1 protein. Skeletal muscle-specific abl …
The biology of Lonp1: More than a mitochondrial protease.
Gibellini L, De Gaetano A, Mandrioli M, Van Tongeren E, Bortolotti CA, Cossarizza A, Pinti M. Gibellini L, et al. Int Rev Cell Mol Biol. 2020;354:1-61. doi: 10.1016/bs.ircmb.2020.02.005. Epub 2020 Mar 25. Int Rev Cell Mol Biol. 2020. PMID: 32475470 Review.
Initially discovered as a protease responsible for degradation of misfolded or damaged proteins, the mitochondrial Lon protease (Lonp1) turned out to be a multifaceted enzyme, that displays at least three different functions (proteolysis, chaperone activity, binding of mtD …
Initially discovered as a protease responsible for degradation of misfolded or damaged proteins, the mitochondrial Lon protease (Lonp1 …
Roles of LonP1 in Oral-Maxillofacial Developmental Defects and Tumors: A Novel Insight.
Ma H, Chen W, Fan W, He H, Huang F. Ma H, et al. Int J Mol Sci. 2022 Nov 2;23(21):13370. doi: 10.3390/ijms232113370. Int J Mol Sci. 2022. PMID: 36362158 Free PMC article. Review.
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). ...Greater research should therefore be conducted on the diagnosis …
LonP1 may therefore be a promising anticancer therapy target. Mutations in LONP1 have been found to be involved in the etiolog …
LONP1 downregulation with ageing contributes to osteoarthritis via mitochondrial dysfunction.
He Y, Ding Q, Chen W, Lin C, Ge L, Ying C, Xu K, Wu Z, Xu L, Ran J, Chen W, Wu L. He Y, et al. Free Radic Biol Med. 2022 Oct;191:176-190. doi: 10.1016/j.freeradbiomed.2022.08.038. Epub 2022 Sep 3. Free Radic Biol Med. 2022. PMID: 36064070
In this study, we found that the expression of Lon protease 1 (LONP1), a mitochondrial protease, was decreased in human OA cartilage and in ageing rat chondrocytes. ...Antioxidant therapy with resveratrol suppressed oxidative stress and MAPK pathway activation induced by …
In this study, we found that the expression of Lon protease 1 (LONP1), a mitochondrial protease, was decreased in human OA cartilage …
Decreased LONP1 expression contributes to DNA damage and meiotic defects in oocytes.
Liu C, Xu M, Guan Y, Li L, Liu W, Guo B, Sheng X, Zhang Y, Zhou J, Zhen X, Yan G, Sun H, Ding L. Liu C, et al. Mol Reprod Dev. 2023 Jun;90(6):358-368. doi: 10.1002/mrd.23694. Epub 2023 Jul 1. Mol Reprod Dev. 2023. PMID: 37392095
In addition, LONP1 downregulation increased oocyte DNA damage. Moreover, we demonstrated that splicing factor proline and glutamine rich directly interacts with LONP1 and mediate the effect of LONP1 depletion on meiotic progression in oocytes. In summary, our …
In addition, LONP1 downregulation increased oocyte DNA damage. Moreover, we demonstrated that splicing factor proline and glutamine r …
Mitochondrial LonP1 protects cardiomyocytes from ischemia/reperfusion injury in vivo.
Venkatesh S, Li M, Saito T, Tong M, Rashed E, Mareedu S, Zhai P, Bárcena C, López-Otín C, Yehia G, Sadoshima J, Suzuki CK. Venkatesh S, et al. J Mol Cell Cardiol. 2019 Mar;128:38-50. doi: 10.1016/j.yjmcc.2018.12.017. Epub 2019 Jan 6. J Mol Cell Cardiol. 2019. PMID: 30625302
In contrast, haploinsufficiency of LonP1 (LONP1(+/-)) abrogated IPC-mediated cardioprotection. Furthermore, LONP1(+/-) mice showed significantly increased infarct size after I/R injury, whereas mice with 3-4 fold cardiac-specific overexpression of LonP1 …
In contrast, haploinsufficiency of LonP1 (LONP1(+/-)) abrogated IPC-mediated cardioprotection. Furthermore, LONP1(+/-) …
191 results