Journal ArticleDOI
Mitochondrial transcription factor A (TFAM): roles in maintenance of mtDNA and cellular functions.
TLDR
Overexpression of human TFAM in mice increases the amount of mitochondrial DNA and dramatically ameliorates the cardiac dysfunctions caused by myocardial infarction.About:
This article is published in Mitochondrion.The article was published on 2007-02-01. It has received 333 citations till now. The article focuses on the topics: TFAM & Mitochondrial fission.read more
Citations
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Journal ArticleDOI
New mitochondrial DNA synthesis enables NLRP3 inflammasome activation
Zhenyu Zhong,Shuang Liang,Elsa Sanchez-Lopez,Feng He,Shabnam Shalapour,Xue Jia Lin,Xue Jia Lin,Jerry Wong,Siyuan Ding,Siyuan Ding,Ekihiro Seki,Bernd Schnabl,Andrea L. Hevener,Harry B. Greenberg,Harry B. Greenberg,Tatiana Kisseleva,Michael Karin +16 more
TL;DR: It is shown that the synthesis of mitochondrial DNA (mtDNA), induced after the engagement of Toll-like receptors, is crucial for NLRP3 signalling, and the dependence on CMPK2 catalytic activity provides opportunities for more effective control ofNLRP3 inflammasome-associated diseases.
Journal ArticleDOI
Microglial cell dysregulation in brain aging and neurodegeneration
TL;DR: Data is discussed suggesting that mitochondrial and endolysosomal dysfunction could at least partially mediate age-associated microglial cell changes, and, together with the impairment of the TGFβ1-Smad3 pathway, could result in the reduction of protective activation and the facilitation of cytotoxic activation of microglia, resulting in the promotion of neurodegenerative diseases.
Journal ArticleDOI
Mitochondrial transcription factor A regulates mitochondrial transcription initiation, DNA packaging, and genome copy number.
TL;DR: An emerging view of TFAM is described as a multipurpose coordinator of mtDNA transactions, with direct consequences for the maintenance of gene expression and genome copy number.
Journal ArticleDOI
The causes of cancer revisited: "mitochondrial malignancy" and ROS-induced oncogenic transformation - why mitochondria are targets for cancer therapy.
Stephen John Ralph,Sara Rodríguez-Enríquez,Jiri Neuzil,Jiri Neuzil,Emma Saavedra,Rafael Moreno-Sánchez +5 more
TL;DR: Evidence highlighting the role of mitochondria as drivers of elevated ROS production during malignant transformation and hence, their potential as targets for cancer therapy is focused on.
Journal ArticleDOI
Peroxisome Proliferator-activated Receptor γ Co-activator 1α (PGC-1α) and Sirtuin 1 (SIRT1) Reside in Mitochondria: POSSIBLE DIRECT FUNCTION IN MITOCHONDRIAL BIOGENESIS*
Katia Aquilano,Paola Vigilanza,Sara Baldelli,Beatrice Pagliei,Giuseppe Rotilio,Maria Rosa Ciriolo +5 more
TL;DR: The results obtained suggest that in mitochondria PGC-1alpha and SIRT1 may function as their nuclear counterparts and represent the genuine factors mediating the cross-talk between nuclear and mitochondrial genome.
References
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Journal ArticleDOI
Dilated Cardiomyopathy and Neonatal Lethality in Mutant Mice Lacking Manganese Superoxide Dismutase
Yibing Li,Ting-Ting Huang,Elaine J. Carlson,Simon Melov,Philip C. Ursell,Jean L. Olson,Linda Noble,Midori P. Yoshimura,Christoph N. Berger,Christoph N. Berger,Pak H. Chan,Douglas C. Wallace,Charles J. Epstein +12 more
TL;DR: Cytochemical analysis revealed a severe reduction in succinate dehydrogenase and aconitase activities in the heart and, to a lesser extent, in other organs, which indicates that MnSOD is required for normal biological function of tissues by maintaining the integrity of mitochondrial enzymes susceptible to direct inactivation by superoxide.
Journal ArticleDOI
Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice
Nils-Göran Larsson,Jianming Wang,Hans Wilhelmsson,Anders Oldfors,Pierre Rustin,Mark Lewandoski,Gregory S. Barsh,David A. Clayton +7 more
TL;DR: The mouse gene for mitochondrial transcription factor A (Tfam), formerly known as m-mtTFA, is disrupted by gene targetting of loxP-sites followed by cre-mediated excision in vivo and is the first mammalian protein demonstrated to regulate mtDNA copy number in vivo.
Journal ArticleDOI
Mitochondrial DNA maintenance in vertebrates.
TL;DR: Because features of a transcription-primed mechanism appear to be conserved in vertebrates, a general model for initiation of vertebrate heavy-strand DNA synthesis is proposed.
Journal ArticleDOI
Human mitochondrial DNA deletions associated with mutations in the gene encoding Twinkle, a phage T7 gene 4-like protein localized in mitochondria.
Johannes N. Spelbrink,Fang Yuan Li,Valeria Tiranti,Kaisu Nikali,Qiu Ping Yuan,Muhammed Tariq,Sjoerd Wanrooij,Nuria Garrido,Giacomo P. Comi,Lucia Morandi,Lucio Santoro,Antonio Toscano,Gian Maria Fabrizi,Hannu Somer,R Croxen,David Beeson,Joanna Poulton,Anu Suomalainen,Howard T. Jacobs,Massimo Zeviani,Catharina Larsson +20 more
TL;DR: A novel mitochondrial protein, Twinkle, with structural similarity to phage T7 gene 4 primase/helicase and other hexameric ring helicases is reported, inferred to be critical for lifetime maintenance of human mtDNA integrity.
Journal ArticleDOI
Mitochondrial transcription factor A regulates mtDNA copy number in mammals
Mats I. Ekstrand,Maria Falkenberg,Anja Rantanen,Chan Bae Park,Martina Gaspari,Kjell Hultenby,Pierre Rustin,Claes M. Gustafsson,Nils-Göran Larsson +8 more
TL;DR: Genetic evidence for a novel role for TFAM in direct regulation of mt DNA copy number in mammals is provided and it is demonstrated that mtDNA copy number is directly proportional to the total TFAM protein levels also in mouse embryos.