| Inactivity of Peptidase ClpP Causes Primary Accumulation of Mitochondrial Disaggregase ClpX with Its Interacting Nucleoid Proteins, and of mtDNA. | Inactivity of Peptidase ClpP Causes Primary Accumulation of Mitochondrial Disaggregase ClpX with Its Interacting Nucleoid Proteins, and of mtDNA.
Key J, Torres-Odio S, Bach NC, Gispert S, Koepf G, Reichlmeir M, West AP, Prokisch H, Freisinger P, Newman WG, Shalev S, Sieber SA, Wittig I, Auburger G., Free PMC Article | 01/22/2022 |
| Heme-dependent recognition of 5-aminolevulinate synthase by the human mitochondrial molecular chaperone ClpX. | Heme-dependent recognition of 5-aminolevulinate synthase by the human mitochondrial molecular chaperone ClpX.
Nomura K, Kitagawa Y, Aihara M, Ohki Y, Furuyama K, Hirokawa T. | 01/15/2022 |
| Experiencing community and domestic violence is associated with epigenetic changes in DNA methylation of BDNF and CLPX in adolescents. | Experiencing community and domestic violence is associated with epigenetic changes in DNA methylation of BDNF and CLPX in adolescents.
Serpeloni F, Nätt D, Assis SG, Wieling E, Elbert T., Free PMC Article | 01/9/2021 |
| The mutation in CLPX inactivates its ATPase activity, resulting in coassembly of mutant and WT protomers to form an enzyme with reduced activity. The presence of low-activity CLPX increases the posttranslational stability of ALAS, causing increased ALAS protein and ALA levels, leading to abnormal accumulation of PPIX. | Mutation in human CLPX elevates levels of δ-aminolevulinate synthase and protoporphyrin IX to promote erythropoietic protoporphyria.
Yien YY, Ducamp S, van der Vorm LN, Kardon JR, Manceau H, Kannengiesser C, Bergonia HA, Kafina MD, Karim Z, Gouya L, Baker TA, Puy H, Phillips JD, Nicolas G, Paw BH., Free PMC Article | 06/9/2018 |
| Data suggest that tumors exploit ClpXP-directed proteostasis to maintain mitochondrial bioenergetics, buffer oxidative stress, and enable metastatic competence. | The Mitochondrial Unfoldase-Peptidase Complex ClpXP Controls Bioenergetics Stress and Metastasis.
Seo JH, Rivadeneira DB, Caino MC, Chae YC, Speicher DW, Tang HY, Vaira V, Bosari S, Palleschi A, Rampini P, Kossenkov AV, Languino LR, Altieri DC., Free PMC Article | 06/24/2017 |
| results illustrate that ClpX overexpression is a good and simple model to study the underlying mechanisms of the UPRmt in mammalian cells. | ClpX stimulates the mitochondrial unfolded protein response (UPRmt) in mammalian cells.
Al-Furoukh N, Ianni A, Nolte H, Hölper S, Krüger M, Wanrooij S, Braun T. | 12/5/2015 |
| Optical trapping to assay single-molecule ClpXP unfolding and translocation of substrates consisting of domains with varying stabilities and sequences; find that ClpXP unfolds most domains by a single pathway, with kinetics that depend on the native fold and structural stability. | Stochastic but highly coordinated protein unfolding and translocation by the ClpXP proteolytic machine.
Cordova JC, Olivares AO, Shin Y, Stinson BM, Calmat S, Schmitz KR, Aubin-Tam ME, Baker TA, Lang MJ, Sauer RT., Free PMC Article | 02/7/2015 |
| Walker B mutation in human CLPX exhibits improved interaction with the model unfolded substrate casein and several putative physiological substrates in vitro. | Substrate recognition and processing by a Walker B mutant of the human mitochondrial AAA+ protein CLPX.
Lowth BR, Kirstein-Miles J, Saiyed T, Brötz-Oesterhelt H, Morimoto RI, Truscott KN, Dougan DA. | 12/22/2012 |
| human ClpX, a novel mtDNA regulator, maintains mtDNA nucleoid distribution through TFAM function as a chaperone rather than as a protease and its involvement in mtDNA segregation. | Maintenance of mitochondrial genome distribution by mitochondrial AAA+ protein ClpX.
Kasashima K, Sumitani M, Endo H. | 12/8/2012 |
| Observational study of gene-disease association. (HuGE Navigator) | Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression.
Hendrickson SL, Lautenberger JA, Chinn LW, Malasky M, Sezgin E, Kingsley LA, Goedert JJ, Kirk GD, Gomperts ED, Buchbinder SP, Troyer JL, O'Brien SJ., Free PMC Article | 12/5/2010 |
| Results reveal that the ssrA tag interacts with different loops that form the top, middle, and lower portions of the central channel of the ClpX hexamer. | Diverse pore loops of the AAA+ ClpX machine mediate unassisted and adaptor-dependent recognition of ssrA-tagged substrates.
Martin A, Baker TA, Sauer RT., Free PMC Article | 01/21/2010 |
| hClpX can regulate the appearance of hClpP peptidase activity in mitochondria and might affect the nature of the degradation products released during ATP-dependent proteolytic cycles | Human mitochondrial ClpP is a stable heptamer that assembles into a tetradecamer in the presence of ClpX.
Kang SG, Dimitrova MN, Ortega J, Ginsburg A, Maurizi MR. | 01/21/2010 |