Structure-Based Design of Selective LONP1 Inhibitors for Probing In Vitro Biology.
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DOI: 10.1021/acs.jmedchem.0c02152
Abstract: LONP1 is an AAA+ protease that maintains mitochondrial homeostasis by removing damaged or misfolded proteins. Elevated activity and expression of LONP1 promotes cancer cell proliferation and resistance to apoptosis-inducing reagents. Despite the importance of LONP1… read more here.
Keywords: lonp1 inhibitors; structure based; biology; lonp1 ... See more keywords
Bi-allelic mutations of LONP1 encoding the mitochondrial LonP1 protease cause pyruvate dehydrogenase deficiency and profound neurodegeneration with progressive cerebellar atrophy
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DOI: 10.1093/hmg/ddy351
Abstract: LonP1 is crucial for maintaining mitochondrial proteostasis and mitigating cell stress. We identified a novel homozygous missense LONP1 variant, c.2282 C > T, (p.Pro761Leu), by whole-exome and Sanger sequencing in two siblings born to healthy consanguineous parents.… read more here.
Keywords: progressive cerebellar; cerebellar atrophy; pyruvate dehydrogenase; lonp1 ... See more keywords
Global Proteome of LonP1+/− Mouse Embryonal Fibroblasts Reveals Impact on Respiratory Chain, but No Interdependence between Eral1 and Mitoribosomes
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DOI: 10.3390/ijms20184523
Abstract: Research on healthy aging shows that lifespan reductions are often caused by mitochondrial dysfunction. Thus, it is very interesting that the deletion of mitochondrial matrix peptidase LonP1 was observed to abolish embryogenesis, while deletion of… read more here.
Keywords: lonp1; mouse embryonal; proteome lonp1; global proteome ... See more keywords
Roles of LonP1 in Oral-Maxillofacial Developmental Defects and Tumors: A Novel Insight
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DOI: 10.3390/ijms232113370
Abstract: 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… read more here.
Keywords: maxillofacial developmental; oral maxillofacial; lonp1 oral; lonp1 ... See more keywords