Gene Symbol: Tfam
Description: transcription factor A, mitochondrial
Alias: AI661103, Hmgts, mtTFA, tsHMG, transcription factor A, mitochondrial, TS-HMG, testis-specific HMG-box protein m-tsHMG, testis-specific high mobility group protein
Species: mouse
Products:     Tfam

Top Publications

  1. Xu A, Kaelin C, Morton G, Ogimoto K, Stanhope K, Graham J, et al. Effects of hypothalamic neurodegeneration on energy balance. PLoS Biol. 2005;3:e415 pubmed
    ..These findings provide new insight into the roles of hypothalamic neurons in energy balance regulation, and provide a model for understanding defects in human energy balance associated with neurodegeneration and aging. ..
  2. Noack H, Bednarek T, Heidler J, Ladig R, Holtz J, Szibor M. TFAM-dependent and independent dynamics of mtDNA levels in C2C12 myoblasts caused by redox stress. Biochim Biophys Acta. 2006;1760:141-50 pubmed
    b>TFAM is an essential protein factor for the initiation of transcription of the mtDNA. It also functions as a packaging factor, which stabilizes the mtDNA pool...
  3. Kaufman B, Durisic N, Mativetsky J, Costantino S, Hancock M, Grutter P, et al. The mitochondrial transcription factor TFAM coordinates the assembly of multiple DNA molecules into nucleoid-like structures. Mol Biol Cell. 2007;18:3225-36 pubmed
    ..Here, we show that the mitochondrial transcription factor TFAM, an abundant and highly conserved High Mobility Group box protein, binds DNA cooperatively with nanomolar affinity ..
  4. Good C, Hoffman A, Hoffer B, Chefer V, Shippenberg T, Bäckman C, et al. Impaired nigrostriatal function precedes behavioral deficits in a genetic mitochondrial model of Parkinson's disease. FASEB J. 2011;25:1333-44 pubmed publisher
    ..Deletion of the mitochondrial transcription factor A gene (Tfam) in C57BL6 mouse DA neurons leads to a slowly progressing parkinsonian phenotype in which motor impairment is first ..
  5. Sterky F, Lee S, Wibom R, Olson L, Larsson N. Impaired mitochondrial transport and Parkin-independent degeneration of respiratory chain-deficient dopamine neurons in vivo. Proc Natl Acad Sci U S A. 2011;108:12937-42 pubmed publisher
    ..We also show that anterograde axonal transport of mitochondria is impaired in respiratory chain-deficient DA neurons, leading to a decreased supply of mitochondria to the axonal terminals. ..
  6. Galter D, Pernold K, Yoshitake T, Lindqvist E, Hoffer B, Kehr J, et al. MitoPark mice mirror the slow progression of key symptoms and L-DOPA response in Parkinson's disease. Genes Brain Behav. 2010;9:173-81 pubmed publisher
    The MitoPark mouse, in which the mitochondrial transcription factor Tfam is selectively removed in midbrain dopamine (DA) neurons, is a genetic model for Parkinson's disease (PD) that replicates the slow and progressive development of key ..
  7. Ekstrand M, Terzioglu M, Galter D, Zhu S, Hofstetter C, Lindqvist E, et al. Progressive parkinsonism in mice with respiratory-chain-deficient dopamine neurons. Proc Natl Acad Sci U S A. 2007;104:1325-30 pubmed
    ..knockout mice (termed MitoPark mice), with disruption of the gene for mitochondrial transcription factor A (Tfam) in DA neurons...
  8. Silva J, Kohler M, Graff C, Oldfors A, Magnuson M, Berggren P, et al. Impaired insulin secretion and beta-cell loss in tissue-specific knockout mice with mitochondrial diabetes. Nat Genet. 2000;26:336-40 pubmed tissue-specific disruption of the nuclear gene encoding mitochondrial transcription factor A (Tfam, previously mtTFA; ref. 7) in pancreatic beta-cells...
  9. Wredenberg A, Wibom R, Wilhelmsson H, Graff C, Wiener H, Burden S, et al. Increased mitochondrial mass in mitochondrial myopathy mice. Proc Natl Acad Sci U S A. 2002;99:15066-71 pubmed animal model for mitochondrial myopathy by disrupting the gene for mitochondrial transcription factor A (Tfam) in skeletal muscle of the mouse...

More Information


  1. Viader A, Golden J, Baloh R, Schmidt R, Hunter D, Milbrandt J. Schwann cell mitochondrial metabolism supports long-term axonal survival and peripheral nerve function. J Neurosci. 2011;31:10128-40 pubmed publisher
    ..These mice (Tfam-SCKOs) were produced through the tissue-specific deletion of the mitochondrial transcription factor A gene (Tfam), ..
  2. Larsson N, Wang J, Wilhelmsson H, Oldfors A, Rustin P, Lewandoski M, et al. Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice. Nat Genet. 1998;18:231-6 pubmed
    ..We have disrupted the mouse gene for mitochondrial transcription factor A (Tfam; formerly known as m-mtTFA) by gene targetting of loxP-sites followed by cre-mediated excision in vivo...
  3. Vernochet C, Mourier A, Bezy O, Macotela Y, Boucher J, Rardin M, et al. Adipose-specific deletion of TFAM increases mitochondrial oxidation and protects mice against obesity and insulin resistance. Cell Metab. 2012;16:765-76 pubmed publisher
    ..whole-body metabolism, we have generated a mouse model with disruption of the mitochondrial transcription factor A (TFAM) specifically in fat...
  4. Wang J, Silva J, Gustafsson C, Rustin P, Larsson N. Increased in vivo apoptosis in cells lacking mitochondrial DNA gene expression. Proc Natl Acad Sci U S A. 2001;98:4038-43 pubmed
    ..Apoptosis was studied in embryos with homozygous disruption of the mitochondrial transcription factor A gene (Tfam) and tissue-specific Tfam knockout animals with severe respiratory chain deficiency in the heart...
  5. Sorensen L, Ekstrand M, Silva J, Lindqvist E, Xu B, Rustin P, et al. Late-onset corticohippocampal neurodepletion attributable to catastrophic failure of oxidative phosphorylation in MILON mice. J Neurosci. 2001;21:8082-90 pubmed
    ..The MILON mouse model provides a novel and powerful tool for additional studies of the role for respiratory chain deficiency in neurodegeneration and aging. ..
  6. Parisi M, Clayton D. Similarity of human mitochondrial transcription factor 1 to high mobility group proteins. Science. 1991;252:965-9 pubmed
    ..Human mtTF1 is most closely related to a DNA binding HMG-box region in hUBF, a human protein known to be important for transcription by RNA polymerase I. ..
  7. Ekstrand M, Falkenberg M, Rantanen A, Park C, Gaspari M, Hultenby K, et al. Mitochondrial transcription factor A regulates mtDNA copy number in mammals. Hum Mol Genet. 2004;13:935-44 pubmed
    ..Mitochondrial transcription factor A (TFAM) is essential for human mtDNA transcription and we demonstrate here that it is also a key regulator of mtDNA copy ..
  8. Wang J, Wilhelmsson H, Graff C, Li H, Oldfors A, Rustin P, et al. Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression. Nat Genet. 1999;21:133-7 pubmed manipulate the expression of the gene encoding mitochondrial transcription factor A (Tfam, previously named mtTFA), which regulates transcription and replication of mtDNA...
  9. West A, Khoury Hanold W, Staron M, Tal M, Pineda C, Lang S, et al. Mitochondrial DNA stress primes the antiviral innate immune response. Nature. 2015;520:553-7 pubmed publisher
    ..The abundant mtDNA-binding protein TFAM (transcription factor A, mitochondrial) regulates nucleoid architecture, abundance and segregation...
  10. Julian M, Shao G, Vangundy Z, Papenfuss T, Crouser E. Mitochondrial transcription factor A, an endogenous danger signal, promotes TNF? release via RAGE- and TLR9-responsive plasmacytoid dendritic cells. PLoS ONE. 2013;8:e72354 pubmed publisher
    Mitochondrial transcription factor A (TFAM) is normally bound to and remains associated with mitochondrial DNA (mtDNA) when released from damaged cells...
  11. Gomez Velazquez M, Badia Careaga C, Lechuga Vieco A, Nieto Arellano R, Tena J, Rollán I, et al. CTCF counter-regulates cardiomyocyte development and maturation programs in the embryonic heart. PLoS Genet. 2017;13:e1006985 pubmed publisher
    ..Thus, CTCF mediates local regulatory interactions to coordinate transcriptional programs controlling transitions in morphology and function during heart development. ..
  12. Chin K, Wieslander C, Shi H, Balgobin S, Montoya T, Yanagisawa H, et al. Pelvic Organ Support in Animals with Partial Loss of Fibulin-5 in the Vaginal Wall. PLoS ONE. 2016;11:e0152793 pubmed publisher
    ..The results indicate that vaginal fibulin-5 during development is crucial for baseline pelvic organ support and is also important for protection and recovery from parturition- and elastase-induced prolapse. ..
  13. Bogenhagen D, Martin D, Koller A. Initial steps in RNA processing and ribosome assembly occur at mitochondrial DNA nucleoids. Cell Metab. 2014;19:618-29 pubmed publisher
    ..These results provide insight into the landscape of binding events required for mitochondrial ribosome assembly and firmly establish the mtDNA nucleoid as a control center for mitochondrial biogenesis. ..
  14. Calo E, Quintero Estades J, Danielian P, Nedelcu S, Berman S, Lees J. Rb regulates fate choice and lineage commitment in vivo. Nature. 2010;466:1110-4 pubmed publisher
    ..Here we use mouse models to address this hypothesis in mesenchymal tissue development and tumorigenesis. Our data show that Rb status plays a key role in establishing fate choice between bone and brown adipose tissue in vivo. ..
  15. Clement A, Mitchelmore C, Andersson D, Asuni A. Cerebrospinal fluid neurofilament light chain as a biomarker of neurodegeneration in the Tg4510 and MitoPark mouse models. Neuroscience. 2017;354:101-109 pubmed publisher
  16. Miyazaki T, Iwasawa M, Nakashima T, Mori S, Shigemoto K, Nakamura H, et al. Intracellular and extracellular ATP coordinately regulate the inverse correlation between osteoclast survival and bone resorption. J Biol Chem. 2012;287:37808-23 pubmed publisher
    ..Severe ATP depletion, caused by disrupting mitochondrial transcription factor A (Tfam) gene, leads to increased bone-resorbing activity despite accelerated apoptosis...
  17. Baris O, Klose A, Kloepper J, Weiland D, Neuhaus J, Schauen M, et al. The mitochondrial electron transport chain is dispensable for proliferation and differentiation of epidermal progenitor cells. Stem Cells. 2011;29:1459-68 pubmed publisher
    ..This was achieved using a loxP-flanked mitochondrial transcription factor A (Tfam) allele in conjunction with a keratin 14 Cre transgene...
  18. Kloepper J, Baris O, Reuter K, Kobayashi K, Weiland D, Vidali S, et al. Mitochondrial function in murine skin epithelium is crucial for hair follicle morphogenesis and epithelial-mesenchymal interactions. J Invest Dermatol. 2015;135:679-689 pubmed publisher development by selectively deleting intraepithelial mtDNA in mice by ablating a key maintenance factor (Tfam(EKO)), which induces loss of function of the electron transport chain (ETC)...
  19. Woo D, Green P, Santos J, D Souza A, Walther Z, Martin W, et al. Mitochondrial genome instability and ROS enhance intestinal tumorigenesis in APC(Min/+) mice. Am J Pathol. 2012;180:24-31 pubmed publisher
    ..Mitochondrial transcription factor A (TFAM) is required for expression and maintenance of mtDNA...
  20. Kopek B, Shtengel G, Xu C, Clayton D, Hess H. Correlative 3D superresolution fluorescence and electron microscopy reveal the relationship of mitochondrial nucleoids to membranes. Proc Natl Acad Sci U S A. 2012;109:6136-41 pubmed publisher
  21. Saleem A, Hood D. Acute exercise induces tumour suppressor protein p53 translocation to the mitochondria and promotes a p53-Tfam-mitochondrial DNA complex in skeletal muscle. J Physiol. 2013;591:3625-36 pubmed publisher
    ..Exercise concomitantly increased the mRNA content of nuclear-encoded (PGC-1?, Tfam, NRF-1, COX-IV, citrate synthase) and mtDNA-encoded (COX-I) genes in the AE group, and further by ?5-fold in the ..
  22. Gordon R, Neal M, Luo J, Langley M, Harischandra D, Panicker N, et al. Prokineticin-2 upregulation during neuronal injury mediates a compensatory protective response against dopaminergic neuronal degeneration. Nat Commun. 2016;7:12932 pubmed publisher
    ..Collectively, our results identify a paradigm for compensatory neuroprotective PK2 signalling in nigral dopaminergic neurons that could have important therapeutic implications for PD. ..
  23. Pierce A, Xu A. De novo neurogenesis in adult hypothalamus as a compensatory mechanism to regulate energy balance. J Neurosci. 2010;30:723-30 pubmed publisher
    ..It further suggests that de novo neurogenesis might serve as a compensatory mechanism contributing to the plastic control of energy balance in response to environmental and physiologic insults. ..
  24. Olofsson L, Pierce A, Xu A. Functional requirement of AgRP and NPY neurons in ovarian cycle-dependent regulation of food intake. Proc Natl Acad Sci U S A. 2009;106:15932-7 pubmed publisher
    ..This study indicates that neurons coexpressing AgRP and NPY are functionally required for the cyclic changes in feeding across estrous cycle and that AgRP and NPY neurons are essential mediators of estrogen's anorexigenic function. ..
  25. Long J, Badal S, Ye Z, Wang Y, Ayanga B, Galvan D, et al. Long noncoding RNA Tug1 regulates mitochondrial bioenergetics in diabetic nephropathy. J Clin Invest. 2016;126:4205-4218 pubmed publisher
    ..These findings indicate that a direct interaction between PGC-1? and Tug1 modulates mitochondrial bioenergetics in podocytes in the diabetic milieu. ..
  26. Duvezin Caubet S, Jagasia R, Wagener J, Hofmann S, Trifunovic A, Hansson A, et al. Proteolytic processing of OPA1 links mitochondrial dysfunction to alterations in mitochondrial morphology. J Biol Chem. 2006;281:37972-9 pubmed
    ..Furthermore, processed OPA1 was observed in heart tissue derived from heart-specific TFAM knock-out mice suffering from mitochondrial cardiomyopathy and in skeletal muscles from patients suffering from ..
  27. Chandrasekaran K, Anjaneyulu M, Inoue T, Choi J, Sagi A, Chen C, et al. Mitochondrial transcription factor A regulation of mitochondrial degeneration in experimental diabetic neuropathy. Am J Physiol Endocrinol Metab. 2015;309:E132-41 pubmed publisher
    ..Mitochondrial transcription factor A (TFAM) wraps mtDNA and promotes mtDNA replication and transcription...
  28. Nisoli E, Falcone S, Tonello C, Cozzi V, Palomba L, Fiorani M, et al. Mitochondrial biogenesis by NO yields functionally active mitochondria in mammals. Proc Natl Acad Sci U S A. 2004;101:16507-12 pubmed
    ..We conclude that NO/cGMP stimulates mitochondrial biogenesis, both in vitro and in vivo, and that this stimulation is associated with increased mitochondrial function, resulting in enhanced formation of ATP. ..
  29. Iacovelli J, Zhao C, Wolkow N, Veldman P, Gollomp K, Ojha P, et al. Generation of Cre transgenic mice with postnatal RPE-specific ocular expression. Invest Ophthalmol Vis Sci. 2011;52:1378-83 pubmed publisher
    ..These mice should facilitate study of the multifunctional RPE and the generation of mouse models of human retinal disease. ..
  30. Blandini F, Armentero M. Animal models of Parkinson's disease. FEBS J. 2012;279:1156-66 pubmed publisher
    ..Therefore, until the 'perfect' model is developed, indications to use one model or another will depend on the specific objectives that are being pursued...
  31. Viader A, Wright Jin E, Vohra B, Heuckeroth R, Milbrandt J. Differential regional and subtype-specific vulnerability of enteric neurons to mitochondrial dysfunction. PLoS ONE. 2011;6:e27727 pubmed publisher
    ..abnormalities in the ENS influence enteric neurodegeneration and affect intestinal function, we generated mice (Tfam-ENSKOs) with impaired mitochondrial metabolism in enteric neurons and glia through the targeted deletion of the ..
  32. Masand R, Paulo E, Wu D, Wang Y, Swaney D, Jimenez Morales D, et al. Proteome Imbalance of Mitochondrial Electron Transport Chain in Brown Adipocytes Leads to Metabolic Benefits. Cell Metab. 2018;27:616-629.e4 pubmed publisher
    ..Furthermore, reducing mtDNA gene expression directly in BAT by removing mitochondrial transcription factor A (Tfam) in BAT also showed ETC proteome imbalance and the trade-off between BAT thermogenesis and systemic metabolism at ..
  33. Fisher R, Parisi M, Clayton D. Flexible recognition of rapidly evolving promoter sequences by mitochondrial transcription factor 1. Genes Dev. 1989;3:2202-17 pubmed
    ..Flexible DNA sequence recognition by mtTF1, on the other hand, may be a principal facilitating mechanism for rapid control sequence evolution. ..
  34. Chae Y, Chung C, Kim B, Lee M, Lee H. The gene encoding guanidinoacetate methyltransferase (GAMT) maps to human chromosome 19 at band p13.3 and to mouse chromosome 10. Genomics. 1998;49:162-4 pubmed
  35. Thundathil J, Filion F, Smith L. Molecular control of mitochondrial function in preimplantation mouse embryos. Mol Reprod Dev. 2005;71:405-13 pubmed
  36. Kühl I, Miranda M, Atanassov I, Kuznetsova I, Hinze Y, Mourier A, et al. Transcriptomic and proteomic landscape of mitochondrial dysfunction reveals secondary coenzyme Q deficiency in mammals. elife. 2017;6: pubmed publisher
  37. Stiles A, Simon M, Stover A, Eftekharian S, Khanlou N, Wang H, et al. Mutations in TFAM, encoding mitochondrial transcription factor A, cause neonatal liver failure associated with mtDNA depletion. Mol Genet Metab. 2016;119:91-9 pubmed publisher
    ..533C>T; p.Pro178Leu) in mitochondrial transcription factor A (TFAM) segregating in a consanguineous kindred of Colombian-Basque descent in which two siblings presented with IUGR, ..
  38. Carmona M, Iglesias R, Obregon M, Darlington G, Villarroya F, Giralt M. Mitochondrial biogenesis and thyroid status maturation in brown fat require CCAAT/enhancer-binding protein alpha. J Biol Chem. 2002;277:21489-98 pubmed
  39. Goto A, Matsushima Y, Kadowaki T, Kitagawa Y. Drosophila mitochondrial transcription factor A (d-TFAM) is dispensable for the transcription of mitochondrial DNA in Kc167 cells. Biochem J. 2001;354:243-8 pubmed
    We have cloned cDNA encoding Drosophila mitochondrial (mt) transcription factor A (d-TFAM)...
  40. Tham E, Wang J, Piehl F, Weber G. Upregulation of VEGF-A without angiogenesis in a mouse model of dilated cardiomyopathy caused by mitochondrial dysfunction. J Histochem Cytochem. 2002;50:935-44 pubmed
    ..Despite the striking upregulation of VEGF-A, there was no increase in capillary density in the knockout hearts. This study suggests that a disturbance in angiogenesis may contribute to the pathogenesis of DCM. ..
  41. Rantanen A, Jansson M, Oldfors A, Larsson N. Downregulation of Tfam and mtDNA copy number during mammalian spermatogenesis. Mamm Genome. 2001;12:787-92 pubmed
    Mitochondrial transcription factor A (Tfam) is required for mtDNA maintenance, and mitochondrial Tfam protein levels directly affect mtDNA copy number...
  42. Hamanaka R, Weinberg S, Reczek C, Chandel N. The Mitochondrial Respiratory Chain Is Required for Organismal Adaptation to Hypoxia. Cell Rep. 2016;15:451-459 pubmed publisher
    ..Therefore, we conditionally deleted expression of TFAM in mouse epidermal keratinocytes...
  43. Cong L, Muir E, Chen C, Qian Y, Liu J, Biju K, et al. Multimodal MRI Evaluation of the MitoPark Mouse Model of Parkinson's Disease. PLoS ONE. 2016;11:e0151884 pubmed publisher
    ..PD), has a dopaminergic neuron-specific knock-out that inactivates the mitochondrial transcription factor A (Tfam), a protein essential for mitochondrial DNA expression and maintenance...
  44. Kukat C, Davies K, Wurm C, Spåhr H, Bonekamp N, Kühl I, et al. Cross-strand binding of TFAM to a single mtDNA molecule forms the mitochondrial nucleoid. Proc Natl Acad Sci U S A. 2015;112:11288-93 pubmed publisher
    Mammalian mitochondrial DNA (mtDNA) is packaged by mitochondrial transcription factor A (TFAM) into mitochondrial nucleoids that are of key importance in controlling the transmission and expression of mtDNA...
  45. Gellhaar S, Marcellino D, Abrams M, Galter D. Chronic L-DOPA induces hyperactivity, normalization of gait and dyskinetic behavior in MitoPark mice. Genes Brain Behav. 2015;14:260-70 pubmed publisher
  46. Weinberg F, Hamanaka R, Wheaton W, Weinberg S, Joseph J, Lopez M, et al. Mitochondrial metabolism and ROS generation are essential for Kras-mediated tumorigenicity. Proc Natl Acad Sci U S A. 2010;107:8788-93 pubmed publisher
    ..Furthermore, disruption of mitochondrial function by loss of the mitochondrial transcription factor A (TFAM) gene reduced tumorigenesis in an oncogenic Kras-driven mouse model of lung cancer...
  47. Koh E, Park J, Park H, Jeon M, Ryu J, Kim M, et al. Essential role of mitochondrial function in adiponectin synthesis in adipocytes. Diabetes. 2007;56:2973-81 pubmed
    ..Impaired mitochondrial function activates a series of mechanisms involving ER stress, JNK, and ATF3 to decrease adiponectin synthesis. ..
  48. Lee K, Yang S, Kim Y, Choi Y, Nam J, Choi C, et al. Disruption of the cereblon gene enhances hepatic AMPK activity and prevents high-fat diet-induced obesity and insulin resistance in mice. Diabetes. 2013;62:1855-64 pubmed publisher
    ..These results provide the first in vivo evidence that Crbn is a negative modulator of AMPK, which suggests that Crbn may be a potential target for metabolic disorders of the liver. ..
  49. Larsson N, Barsh G, Clayton D. Structure and chromosomal localization of the mouse mitochondrial transcription factor A gene (Tfam). Mamm Genome. 1997;8:139-40 pubmed
  50. Yamamoto H, Morino K, Nishio Y, Ugi S, Yoshizaki T, Kashiwagi A, et al. MicroRNA-494 regulates mitochondrial biogenesis in skeletal muscle through mitochondrial transcription factor A and Forkhead box j3. Am J Physiol Endocrinol Metab. 2012;303:E1419-27 pubmed publisher
    ..Furthermore, the expression of predicted target genes for miR-494, including mitochondrial transcription factor A (mtTFA) and Forkhead box j3 (Foxj3), was posttranscriptionally increased during myogenic differentiation...
  51. Andreeva L, Hiller B, Kostrewa D, Lässig C, de Oliveira Mann C, Jan Drexler D, et al. cGAS senses long and HMGB/TFAM-bound U-turn DNA by forming protein-DNA ladders. Nature. 2017;549:394-398 pubmed publisher
    ..Remarkably, bacterial and mitochondrial nucleoid proteins HU and mitochondrial transcription factor A (TFAM), as well as high-mobility group box 1 protein (HMGB1), can strongly stimulate long DNA sensing by cGAS...
  52. Brown T, Tkachuk A, Clayton D. Mitochondrial Transcription Factor A (TFAM) Binds to RNA Containing 4-Way Junctions and Mitochondrial tRNA. PLoS ONE. 2015;10:e0142436 pubmed publisher
    ..These structures are highly condensed by the DNA packaging protein, mitochondrial Transcription Factor A (TFAM)...
  53. Hokari M, Kuroda S, Kinugawa S, Ide T, Tsutsui H, Iwasaki Y. Overexpression of mitochondrial transcription factor A (TFAM) ameliorates delayed neuronal death due to transient forebrain ischemia in mice. Neuropathology. 2010;30:401-7 pubmed publisher
    Mitochondrial transcription factor A (TFAM) is an important regulator to maintain mitochondrial DNA copy number. However, no studies have denoted its roles in cerebral ischemia...
  54. Boissonneault G, Lau Y. A testis-specific gene encoding a nuclear high-mobility-group box protein located in elongating spermatids. Mol Cell Biol. 1993;13:4323-30 pubmed
    ..These results suggest that this novel HMG box protein gene may be involved in the regulation of gene expression of the haploid male genome. The gene from which the cDNA was derived has been termed testis-specific HMG (tsHMG).
  55. Zobeley E, Sufalko D, Adkins S, Burmeister M. Fine genetic and comparative mapping of the deafness mutation Ames waltzer on mouse chromosome 10. Genomics. 1998;50:260-6 pubmed
    ..In addition, nine different genes (Ank3, Bcr, Gnaz, Tfam, Mif, Mmp11, Dcoh, Pyp, and Gstt2) were mapped and eliminated genetically as candidate genes for av...
  56. Li H, Wang J, Wilhelmsson H, Hansson A, Thoren P, Duffy J, et al. Genetic modification of survival in tissue-specific knockout mice with mitochondrial cardiomyopathy. Proc Natl Acad Sci U S A. 2000;97:3467-72 pubmed
    ..The gene for mouse mitochondrial transcription factor A, Tfam (also called mtTFA), a nucleus-encoded key regulator of mtDNA expression, was targeted with loxP sites (Tfam(loxP)) and disrupted in ..
  57. Wredenberg A, Freyer C, Sandstrom M, Katz A, Wibom R, Westerblad H, et al. Respiratory chain dysfunction in skeletal muscle does not cause insulin resistance. Biochem Biophys Res Commun. 2006;350:202-7 pubmed
    ..In summary, our findings indicate that mitochondrial dysfunction in skeletal muscle is not a primary etiological event in DM2. ..
  58. Shi C, Xu G, Yang L, Fu Z, Chen L, Fu H, et al. Overexpression of TFAM protects 3T3-L1 adipocytes from NYGGF4 (PID1) overexpression-induced insulin resistance and mitochondrial dysfunction. Cell Biochem Biophys. 2013;66:489-97 pubmed publisher
    ..underlying NYGGF4-induced IR by investigating the effect of overexpressing mitochondrial transcription factor A (TFAM), which is essential for mitochondrial DNA transcription and replication, on NYGGF4-induced IR and mitochondrial ..
  59. Branch S, Chen C, Sharma R, Lechleiter J, Li S, Beckstead M. Dopaminergic Neurons Exhibit an Age-Dependent Decline in Electrophysiological Parameters in the MitoPark Mouse Model of Parkinson's Disease. J Neurosci. 2016;36:4026-37 pubmed publisher
    ..Because PD is a progressive disease with a long asymptomatic phase, identification of early functional adaptations could lay the groundwork to test therapeutic interventions that halt or reverse disease progression. ..
  60. Hansson A, Hance N, Dufour E, Rantanen A, Hultenby K, Clayton D, et al. A switch in metabolism precedes increased mitochondrial biogenesis in respiratory chain-deficient mouse hearts. Proc Natl Acad Sci U S A. 2004;101:3136-41 pubmed
    ..The tissue-specific mitochondrial transcription factor A (Tfam) knockout mice of this study displayed a progressive heart phenotype with depletion of mtDNA and an accompanying ..
  61. Hamanaka R, Glasauer A, Hoover P, Yang S, Blatt H, Mullen A, et al. Mitochondrial reactive oxygen species promote epidermal differentiation and hair follicle development. Sci Signal. 2013;6:ra8 pubmed publisher
    ..differentiation by generating mice with a keratinocyte-specific deficiency in mitochondrial transcription factor A (TFAM), which is required for the transcription of mitochondrial genes encoding electron transport chain subunits...
  62. Li X, Redus L, Chen C, Martinez P, Strong R, Li S, et al. Cognitive dysfunction precedes the onset of motor symptoms in the MitoPark mouse model of Parkinson's disease. PLoS ONE. 2013;8:e71341 pubmed publisher
    ..Together, these data establish that mild cognitive impairment precedes the loss of motor function in a novel rodent model of PD, which may provide unique opportunities for therapeutic development. ..
  63. Viader A, Sasaki Y, Kim S, Strickland A, Workman C, Yang K, et al. Aberrant Schwann cell lipid metabolism linked to mitochondrial deficits leads to axon degeneration and neuropathy. Neuron. 2013;77:886-98 pubmed publisher
    ..Here, we investigate a mouse model of peripheral neuropathy secondary to SC mitochondrial dysfunction (Tfam-SCKOs)...
  64. Gineste C, Hernandez A, Ivarsson N, Cheng A, Naess K, Wibom R, et al. Cyclophilin D, a target for counteracting skeletal muscle dysfunction in mitochondrial myopathy. Hum Mol Genet. 2015;24:6580-7 pubmed publisher
    ..In a mouse model of lethal mitochondrial myopathy, the muscle-specific Tfam knock-out (KO) mouse, we previously demonstrated an excessive mitochondrial Ca(2+) uptake in isolated muscle fibers ..
  65. Collu Marchese M, Shuen M, Pauly M, Saleem A, Hood D. The regulation of mitochondrial transcription factor A (Tfam) expression during skeletal muscle cell differentiation. Biosci Rep. 2015;35: pubmed publisher
    ..The most important transcriptional activator of the mitochondrial genome is mitochondrial transcription factor A (Tfam); however, the regulation of Tfam expression during muscle differentiation is not known...
  66. Vernochet C, Damilano F, Mourier A, Bezy O, Mori M, Smyth G, et al. Adipose tissue mitochondrial dysfunction triggers a lipodystrophic syndrome with insulin resistance, hepatosteatosis, and cardiovascular complications. FASEB J. 2014;28:4408-19 pubmed publisher
    ..mitochondrial impairment in adipose tissue, we generated mice deficient in mitochondrial transcription factor A (TFAM) in adipocytes by using mice carrying adiponectin-Cre and TFAM floxed alleles...
  67. Beckervordersandforth R, Ebert B, Schäffner I, Moss J, Fiebig C, Shin J, et al. Role of Mitochondrial Metabolism in the Control of Early Lineage Progression and Aging Phenotypes in Adult Hippocampal Neurogenesis. Neuron. 2017;93:560-573.e6 pubmed publisher
    ..Perturbation of mitochondrial complex function by ablation of the mitochondrial transcription factor A (Tfam) reproduces multiple hallmarks of aging in hippocampal neurogenesis, whereas pharmacological enhancement of ..
  68. Milenkovic D, Matic S, Kühl I, Ruzzenente B, Freyer C, Jemt E, et al. TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication. Hum Mol Genet. 2013;22:1983-93 pubmed publisher
    ..Our data thus suggest that the relative levels of abortive D-loop synthesis versus complete mtDNA replication are regulated and may provide a mechanism to control progression to complete mtDNA replication. ..
  69. Westerlund M, Ran C, Borgkvist A, Sterky F, Lindqvist E, Lundströmer K, et al. Lrrk2 and alpha-synuclein are co-regulated in rodent striatum. Mol Cell Neurosci. 2008;39:586-91 pubmed publisher
    ..Because striatal Lrrk2 and alpha-synuclein levels were not changed by dopamine depletion, we conclude that Lrrk2 and alpha-synuclein mRNA levels are possibly co-regulated, but they are not influenced by striatal dopamine levels. ..
  70. Weiland D, Brachvogel B, Hornig Do H, Neuhaus J, Holzer T, Tobin D, et al. Imbalance of Mitochondrial Respiratory Chain Complexes in the Epidermis Induces Severe Skin Inflammation. J Invest Dermatol. 2018;138:132-140 pubmed publisher
  71. Unger E, Piper M, Olofsson L, Xu A. Functional role of c-Jun-N-terminal kinase in feeding regulation. Endocrinology. 2010;151:671-82 pubmed publisher
    ..Thus, JNK may integrate diverse metabolic signals and differentially regulate feeding under distinct physiological conditions. ..