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2021 12
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Page 1
Genetics of ovarian insufficiency and defects of folliculogenesis.
França MM, Mendonca BB. França MM, et al. Best Pract Res Clin Endocrinol Metab. 2022 Jan;36(1):101594. doi: 10.1016/j.beem.2021.101594. Epub 2021 Oct 14. Best Pract Res Clin Endocrinol Metab. 2022. PMID: 34794894 Review.
Thirty-four of these genes (AARS2, AIRE, ANTXR1, ATM, BMPR1B, CLPP, CYP17A1, CYP19A1, DCAF17, EIF2B, ERAL1, FANCA, FANCC, FMR1, FOXL2, GALT, GNAS, HARS2, HSD17B4, LARS2, LMNA, MGME1, NBN, PMM2, POLG, PREPL, RCBTB1, RECQL2/3/4, STAR, TWNK, and XRCC4/9) have been linked to s …
Thirty-four of these genes (AARS2, AIRE, ANTXR1, ATM, BMPR1B, CLPP, CYP17A1, CYP19A1, DCAF17, EIF2B, ERAL1, FANCA, FANCC, FMR1, FOXL2 …
A mitochondrial unfolded protein response inhibitor suppresses prostate cancer growth in mice via HSP60.
Kumar R, Chaudhary AK, Woytash J, Inigo JR, Gokhale AA, Bshara W, Attwood K, Wang J, Spernyak JA, Rath E, Yadav N, Haller D, Goodrich DW, Tang DG, Chandra D. Kumar R, et al. J Clin Invest. 2022 Jul 1;132(13):e149906. doi: 10.1172/JCI149906. J Clin Invest. 2022. PMID: 35653190 Free PMC article.
Here we show that the 2 key components of the UPRmt, heat shock protein 60 (HSP60, a mitochondrial chaperonin) and caseinolytic protease P (ClpP, a mitochondrial protease), were required for the development of advanced PCa. HSP60 regulated ClpP expression via c-Myc …
Here we show that the 2 key components of the UPRmt, heat shock protein 60 (HSP60, a mitochondrial chaperonin) and caseinolytic protease P ( …
Ataxia and Hypogonadism: a Review of the Associated Genes and Syndromes.
De Michele G, Maione L, Cocozza S, Tranfa M, Pane C, Galatolo D, De Rosa A, De Michele G, Saccà F, Filla A. De Michele G, et al. Cerebellum. 2024 Apr;23(2):688-701. doi: 10.1007/s12311-023-01549-x. Epub 2023 Mar 30. Cerebellum. 2024. PMID: 36997834 Review.
In the second part, we mention clinical syndromes and genes (POLR3A, CLPP, ERAL1, HARS, HSD17B4, LARS2, TWNK, POLG, ATM, WFS1, PMM2, FMR1) linked to complex phenotypes that include, among other features, ataxia and hypogonadism. ...
In the second part, we mention clinical syndromes and genes (POLR3A, CLPP, ERAL1, HARS, HSD17B4, LARS2, TWNK, POLG, ATM, WFS1, PMM2, …
ATF5-mediated mitochondrial unfolded protein response protects against Pb-induced mitochondria damage in SH-SY5Y cell.
Xu Y, Liu M, Gao S, Li X, Chen J, Ye F. Xu Y, et al. Neurotoxicology. 2024 Dec;105:293-302. doi: 10.1016/j.neuro.2024.11.001. Epub 2024 Nov 14. Neurotoxicology. 2024. PMID: 39547369
In this study, we found that sublethal level exposure of PbAc (2.5 muM) could cause mitochondria damage and then activate mtUPR by promoting the expression of mitochondrial proteases (LonP1 and ClpP), molecular chaperone (HSPA1A). ATF5 mediated mtUPR activation as knocking …
In this study, we found that sublethal level exposure of PbAc (2.5 muM) could cause mitochondria damage and then activate mtUPR by promoting …
The mitochondrial protease ClpP is a druggable target that controls VSMC phenotype by a SIRT1-dependent mechanism.
Paredes F, Williams HC, Liu X, Holden C, Bogan B, Wang Y, Crotty KM, Yeligar SM, Elorza AA, Lin Z, Rezvan A, San Martin A. Paredes F, et al. Redox Biol. 2024 Jul;73:103203. doi: 10.1016/j.redox.2024.103203. Epub 2024 May 21. Redox Biol. 2024. PMID: 38823208 Free PMC article.
Our mechanistic exploration indicates that ClpP activation regulates the VSMC phenotype by modifying the cellular NAD(+)/NADH ratio and activating Sirtuin 1. Our findings reveal the crucial role of mitochondrial proteostasis in the regulation of the VSMC phenotype and prop …
Our mechanistic exploration indicates that ClpP activation regulates the VSMC phenotype by modifying the cellular NAD(+)/NADH ratio a …
Mechanism of allosteric activation in human mitochondrial ClpP protease.
Goncalves MM, Uday AB, Forrester TJB, Currie SQW, Kim AS, Feng Y, Jitkova Y, Velyvis A, Harkness RW, Kimber MS, Schimmer AD, Zeytuni N, Vahidi S. Goncalves MM, et al. Proc Natl Acad Sci U S A. 2025 Apr 22;122(16):e2419881122. doi: 10.1073/pnas.2419881122. Epub 2025 Apr 15. Proc Natl Acad Sci U S A. 2025. PMID: 40232800 Free PMC article.
Here, we present structures of human mitochondrial ClpP in the active extended state, including a pair of structures where ClpP is bound to an active-site inhibitor. ...Our findings link the conformational dynamics of ClpP to its catalytic function and provid …
Here, we present structures of human mitochondrial ClpP in the active extended state, including a pair of structures where ClpP …
Selective DRD2 antagonist and ClpP agonist ONC201 in a recurrent non-midline H3 K27M-mutant glioma cohort.
Odia Y, Hall MD, Cloughesy TF, Wen PY, Arrillaga-Romany I, Daghistani D, Mehta MP, Tarapore RS, Ramage SC, Allen JE. Odia Y, et al. Neuro Oncol. 2024 May 3;26(Supplement_2):S165-S172. doi: 10.1093/neuonc/noae021. Neuro Oncol. 2024. PMID: 38386699 Free PMC article.
Dordaviprone (ONC201) is an oral investigational small molecule, DRD2 antagonist, and ClpP agonist associated with durable responses in recurrent H3 K27M-mutant DMG. Activity of ONC201 in non-midline H3 K27M-mutant diffuse gliomas has not been reported. ...
Dordaviprone (ONC201) is an oral investigational small molecule, DRD2 antagonist, and ClpP agonist associated with durable responses …
New insights into Perrault syndrome, a clinically and genetically heterogeneous disorder.
Faridi R, Rea A, Fenollar-Ferrer C, O'Keefe RT, Gu S, Munir Z, Khan AA, Riazuddin S, Hoa M, Naz S, Newman WG, Friedman TB. Faridi R, et al. Hum Genet. 2022 Apr;141(3-4):805-819. doi: 10.1007/s00439-021-02319-7. Epub 2021 Aug 2. Hum Genet. 2022. PMID: 34338890 Free PMC article. Review.
Here, we review the molecular genetics and clinical phenotype of Perrault syndrome and focus on supporting evidence for the eight genes (CLPP, ERAL1, GGPS1, HARS2, HSD17B4, LARS2, RMND1, TWNK) associated with Perrault syndrome. ...We also report a new genetic variant of …
Here, we review the molecular genetics and clinical phenotype of Perrault syndrome and focus on supporting evidence for the eight genes ( …
Cervimycin-Resistant Staphylococcus aureus Strains Display Vancomycin-Intermediate Resistant Phenotypes.
Dietrich A, Steffens U, Gajdiss M, Boschert AL, Dröge JK, Szekat C, Sass P, Malik IT, Bornikoel J, Reinke L, Maček B, Franz-Wachtel M, Nieselt K, Harbig T, Scherlach K, Brötz-Oesterhelt H, Hertweck C, Sahl HG, Bierbaum G. Dietrich A, et al. Microbiol Spectr. 2022 Oct 26;10(5):e0256722. doi: 10.1128/spectrum.02567-22. Epub 2022 Sep 29. Microbiol Spectr. 2022. PMID: 36173303 Free PMC article.
Genome sequencing of CmR mutants revealed amino acid exchanges in the essential histidine kinase WalK, the Clp protease proteolytic subunit ClpP or the Clp ATPase ClpC, and the heat shock protein DnaK. Interestingly, all characterized CmR mutants harbored a combination of …
Genome sequencing of CmR mutants revealed amino acid exchanges in the essential histidine kinase WalK, the Clp protease proteolytic subunit …
Knockout Mouse Studies Show That Mitochondrial CLPP Peptidase and CLPX Unfoldase Act in Matrix Condensates near IMM, as Fast Stress Response in Protein Assemblies for Transcript Processing, Translation, and Heme Production.
Key J, Gispert S, Auburger G. Key J, et al. Genes (Basel). 2024 May 27;15(6):694. doi: 10.3390/genes15060694. Genes (Basel). 2024. PMID: 38927630 Free PMC article. Review.
The AAA+ unfoldase CLPX and the peptidase CLPP also act in the matrix, especially during stress periods, but their substrates are poorly defined. Mammalian CLPP deletion triggers infertility, deafness, growth retardation, and cGAS-STING-activated cytosolic innate im …
The AAA+ unfoldase CLPX and the peptidase CLPP also act in the matrix, especially during stress periods, but their substrates are poo …
63 results