Tfam

Summary

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

Top Publications

  1. pmc Effects of hypothalamic neurodegeneration on energy balance
    Allison Wanting Xu
    Department of Genetics, Stanford University School of Medicine, Stanford, California, USA
    PLoS Biol 3:e415. 2005
  2. ncbi TFAM-dependent and independent dynamics of mtDNA levels in C2C12 myoblasts caused by redox stress
    Heiko Noack
    Institute of Pathophysiology, Martin Luther University Halle Wittenberg, ZAMED, Heinrich Damerow Str 1, 06097 Halle, Germany
    Biochim Biophys Acta 1760:141-50. 2006
  3. pmc The mitochondrial transcription factor TFAM coordinates the assembly of multiple DNA molecules into nucleoid-like structures
    Brett A Kaufman
    Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, Montreal, QC, H3A 2B4, Canada
    Mol Biol Cell 18:3225-36. 2007
  4. pmc MitoPark mice mirror the slow progression of key symptoms and L-DOPA response in Parkinson's disease
    D Galter
    Department of Neuroscience, Retzius vag 8, B2 4, Karolinska Institutet, 171 77 Stockholm, Sweden
    Genes Brain Behav 9:173-81. 2010
  5. pmc Increased mitochondrial mass in mitochondrial myopathy mice
    Anna Wredenberg
    Department of Medical Nutrition and Biosciences, Karolinska Institute, Huddinge Hospital, Sweden
    Proc Natl Acad Sci U S A 99:15066-71. 2002
  6. ncbi Mitochondrial transcription factor A regulates mtDNA copy number in mammals
    Mats I Ekstrand
    Department of Medical Nutrition, Karolinska Instititutet, Novum, S 141 86 Stockholm, Sweden
    Hum Mol Genet 13:935-44. 2004
  7. pmc Impaired nigrostriatal function precedes behavioral deficits in a genetic mitochondrial model of Parkinson's disease
    Cameron H Good
    Cellular Neurobiology Branch, National Institute on Drug Abuse Intramural Research Program, National Institutes of Health, U S Department of Health and Human Services, Baltimore, MD 21224, USA
    FASEB J 25:1333-44. 2011
  8. pmc Schwann cell mitochondrial metabolism supports long-term axonal survival and peripheral nerve function
    Andreu Viader
    Department of Genetics, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Neurosci 31:10128-40. 2011
  9. ncbi Late-onset corticohippocampal neurodepletion attributable to catastrophic failure of oxidative phosphorylation in MILON mice
    L Sorensen
    Departments of Medical Nutrition and Biosciences, Karolinska Institutet, Novum, Huddinge Hospital, S 141 86 Huddinge, Sweden
    J Neurosci 21:8082-90. 2001
  10. pmc Increased in vivo apoptosis in cells lacking mitochondrial DNA gene expression
    J Wang
    Department of Molecular Medicine, Karolinska Institutet, L8 02, Karolinska Hospital, S 171 76 Stockholm, Sweden
    Proc Natl Acad Sci U S A 98:4038-43. 2001

Scientific Experts

  • Daniel F Bogenhagen
  • Karim Fifel
  • Fabio Blandini
  • Heiko Noack
  • Satoru Abe
  • D Galter
  • Enzo Nisoli
  • N G Larsson
  • Emma Tham
  • Andreu Viader
  • Anna Wredenberg
  • Robert B Hamanaka
  • Navdeep S Chandel
  • Rolf Wibom
  • Hakan Westerblad
  • Chan Bae Park
  • Lars Olson
  • Maria Falkenberg
  • Jennifer E Kloepper
  • Ayesha Saleem
  • Kevin Y Lee
  • Olivier R Baris
  • Cecile Vernochet
  • Jeffrey Milbrandt
  • Christoph Freyer
  • Chen Zhao
  • Mats I Ekstrand
  • David A Clayton
  • Fredrik H Sterky
  • Andrew A Pierce
  • Allison W Xu
  • Anna Hansson
  • Louise E Olofsson
  • Sarah Y Branch
  • Melania Collu-Marchese
  • J Wang
  • Krish Chandrasekaran
  • A Phillip West
  • Charlotte Gineste
  • Christian Kukat
  • Timothy A Brown
  • Pasi Tavi
  • David A Hood
  • Inge Kühl
  • Gerald S Shadel
  • Kwang Min Lee
  • Desmond J Tobin
  • Rudolf J Wiesner
  • Daniela Weiland
  • Ralf Paus
  • Dusanka Milenkovic
  • Graham Smyth
  • C Ronald Kahn
  • Jeremie Boucher
  • Daniel C Andersson
  • Hirotaka Yamamoto
  • Dong Kyun Woo
  • Benjamin G Kopek
  • Tsuyoshi Miyazaki
  • Joseph D Bruton
  • Kyriakos N Papanicolaou
  • Douglas Vollrath
  • Martina Gaspari
  • Jared Iacovelli
  • J P Silva
  • Cameron H Good
  • Joshua L Dunaief
  • P Rustin
  • Shi Jin Zhang
  • Timothy Wai
  • Elizabeth K Unger
  • Masaaki Hokari
  • Frank Weinberg
  • Eliezer Calo
  • Jan Aydin
  • Eric A Shoubridge
  • Marie Westerlund
  • Eva Lindqvist
  • Brett A Kaufman
  • Eun Hee Koh
  • Aleksandra Trifunovic
  • Stéphane Duvezin-Caubet
  • A Oldfors
  • Allison Wanting Xu
  • Jacob Thundathil
  • H Li
  • Anja Rantanen
  • Kjell Hultenby
  • Claes M Gustafsson
  • Colleen R Reczek

Detail Information

Publications72

  1. pmc Effects of hypothalamic neurodegeneration on energy balance
    Allison Wanting Xu
    Department of Genetics, Stanford University School of Medicine, Stanford, California, USA
    PLoS Biol 3:e415. 2005
    ..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. ncbi TFAM-dependent and independent dynamics of mtDNA levels in C2C12 myoblasts caused by redox stress
    Heiko Noack
    Institute of Pathophysiology, Martin Luther University Halle Wittenberg, ZAMED, Heinrich Damerow Str 1, 06097 Halle, Germany
    Biochim Biophys Acta 1760:141-50. 2006
    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. pmc The mitochondrial transcription factor TFAM coordinates the assembly of multiple DNA molecules into nucleoid-like structures
    Brett A Kaufman
    Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, Montreal, QC, H3A 2B4, Canada
    Mol Biol Cell 18:3225-36. 2007
    ..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. pmc MitoPark mice mirror the slow progression of key symptoms and L-DOPA response in Parkinson's disease
    D Galter
    Department of Neuroscience, Retzius vag 8, B2 4, Karolinska Institutet, 171 77 Stockholm, Sweden
    Genes Brain Behav 9:173-81. 2010
    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 ..
  5. pmc Increased mitochondrial mass in mitochondrial myopathy mice
    Anna Wredenberg
    Department of Medical Nutrition and Biosciences, Karolinska Institute, Huddinge Hospital, Sweden
    Proc Natl Acad Sci U S A 99:15066-71. 2002
    ..an animal model for mitochondrial myopathy by disrupting the gene for mitochondrial transcription factor A (Tfam) in skeletal muscle of the mouse...
  6. ncbi Mitochondrial transcription factor A regulates mtDNA copy number in mammals
    Mats I Ekstrand
    Department of Medical Nutrition, Karolinska Instititutet, Novum, S 141 86 Stockholm, Sweden
    Hum Mol Genet 13:935-44. 2004
    ..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 ..
  7. pmc Impaired nigrostriatal function precedes behavioral deficits in a genetic mitochondrial model of Parkinson's disease
    Cameron H Good
    Cellular Neurobiology Branch, National Institute on Drug Abuse Intramural Research Program, National Institutes of Health, U S Department of Health and Human Services, Baltimore, MD 21224, USA
    FASEB J 25:1333-44. 2011
    ..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 ..
  8. pmc Schwann cell mitochondrial metabolism supports long-term axonal survival and peripheral nerve function
    Andreu Viader
    Department of Genetics, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Neurosci 31:10128-40. 2011
    ..These mice (Tfam-SCKOs) were produced through the tissue-specific deletion of the mitochondrial transcription factor A gene (Tfam), ..
  9. ncbi Late-onset corticohippocampal neurodepletion attributable to catastrophic failure of oxidative phosphorylation in MILON mice
    L Sorensen
    Departments of Medical Nutrition and Biosciences, Karolinska Institutet, Novum, Huddinge Hospital, S 141 86 Huddinge, Sweden
    J Neurosci 21:8082-90. 2001
    ..The MILON mouse model provides a novel and powerful tool for additional studies of the role for respiratory chain deficiency in neurodegeneration and aging...
  10. pmc Increased in vivo apoptosis in cells lacking mitochondrial DNA gene expression
    J Wang
    Department of Molecular Medicine, Karolinska Institutet, L8 02, Karolinska Hospital, S 171 76 Stockholm, Sweden
    Proc Natl Acad Sci U S A 98:4038-43. 2001
    ..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...
  11. pmc Impaired mitochondrial transport and Parkin-independent degeneration of respiratory chain-deficient dopamine neurons in vivo
    Fredrik H Sterky
    Department of Laboratory Medicine, Karolinska Institute, SE 171 77 Stockholm, Sweden
    Proc Natl Acad Sci U S A 108:12937-42. 2011
    ..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...
  12. ncbi Impaired insulin secretion and beta-cell loss in tissue-specific knockout mice with mitochondrial diabetes
    J P Silva
    Department of Molecular Medicine, Karolinska Institutet, Karolinska Hospital, Stockholm, Sweden
    Nat Genet 26:336-40. 2000
    ..by tissue-specific disruption of the nuclear gene encoding mitochondrial transcription factor A (Tfam, previously mtTFA; ref. 7) in pancreatic beta-cells...
  13. pmc Adipose-specific deletion of TFAM increases mitochondrial oxidation and protects mice against obesity and insulin resistance
    Cecile Vernochet
    Section on Integrative Physiology and Metabolism, Joslin Diabetes Center, Harvard Medical School, Boston, MA 02115, USA
    Cell Metab 16:765-76. 2012
    ..whole-body metabolism, we have generated a mouse model with disruption of the mitochondrial transcription factor A (TFAM) specifically in fat...
  14. ncbi Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression
    J Wang
    Department of Molecular Medicine, Center for Molecular Medicine L8 02, Karolinska Hospital, Stockholm, Sweden
    Nat Genet 21:133-7. 1999
    ..to manipulate the expression of the gene encoding mitochondrial transcription factor A (Tfam, previously named mtTFA), which regulates transcription and replication of mtDNA...
  15. ncbi Similarity of human mitochondrial transcription factor 1 to high mobility group proteins
    M A Parisi
    Department of Developmental Biology, Stanford University School of Medicine, CA 94305
    Science 252:965-9. 1991
    ..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...
  16. pmc Progressive parkinsonism in mice with respiratory-chain-deficient dopamine neurons
    Mats I Ekstrand
    Department of Laboratory Medicine, Karolinska Institutet, Karolinska University Hospital, S 14186 Stockholm, Sweden
    Proc Natl Acad Sci U S A 104:1325-30. 2007
    ..knockout mice (termed MitoPark mice), with disruption of the gene for mitochondrial transcription factor A (Tfam) in DA neurons...
  17. ncbi Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice
    N G Larsson
    Department of Molecular Medicine, Center for Molecular Medicine L8 02, Stockholm, Sweden
    Nat Genet 18:231-6. 1998
    ..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...
  18. pmc Disruption of the cereblon gene enhances hepatic AMPK activity and prevents high-fat diet-induced obesity and insulin resistance in mice
    Kwang Min Lee
    School of Life Sciences and Cell Dynamics Research Center and National Leading Research Laboratory for Ion Channels, Gwangju Institute of Science and Technology, Gwangju, Republic of Korea
    Diabetes 62:1855-64. 2013
    ..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...
  19. pmc Mitofusins 1 and 2 are essential for postnatal metabolic remodeling in heart
    Kyriakos N Papanicolaou
    Whitaker Cardiovascular Institute, Boston University School of Medicine, Boston, MA, USA
    Circ Res 111:1012-26. 2012
    ..Mitofusins (Mfn-1 and Mfn-2) are known regulators of mitochondrial networks, but their role during perinatal maturation of the heart has yet to be examined...
  20. pmc Differential regional and subtype-specific vulnerability of enteric neurons to mitochondrial dysfunction
    Andreu Viader
    Department of Genetics, Washington University School of Medicine, St Louis, Missouri, United States of America
    PLoS ONE 6:e27727. 2011
    ..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 ..
  21. pmc Acute exercise induces tumour suppressor protein p53 translocation to the mitochondria and promotes a p53-Tfam-mitochondrial DNA complex in skeletal muscle
    Ayesha Saleem
    School of Kinesiology and Health Science, Muscle Health Research Centre, York University, Toronto, Ontario, Canada
    J Physiol 591:3625-36. 2013
    ..Exercise concomitantly increased the mRNA content of nuclear-encoded (PGC-1α, 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. doi Initial steps in RNA processing and ribosome assembly occur at mitochondrial DNA nucleoids
    Daniel F Bogenhagen
    Department of Pharmacological Sciences, Stony Brook University, Stony Brook, NY 11794 8651, USA Electronic address
    Cell Metab 19:618-29. 2014
    ..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...
  23. doi Loss of dopamine disrupts circadian rhythms in a mouse model of Parkinson's disease
    Karim Fifel
    INSERM 846, Stem Cell and Brain Research Institute, Department of Chronobiology, 18 Avenue du Doyen Lepine, 69500 Bron, France Laboratory of Neurophysiology, Molecular Cell Biology Department, Leiden University Medical Center, PO Box 9600 Mailbox S5 P 2300 RC Leiden, The Netherlands Electronic address
    Neurobiol Dis 71:359-69. 2014
    ..behaviors in a transgenic MitoPark mouse model with selective inactivation of mitochondrial transcription factor A (Tfam) resulting in a slow and progressive degeneration of DA neurons in midbrain structures...
  24. doi Mitochondrial function in murine skin epithelium is crucial for hair follicle morphogenesis and epithelial-mesenchymal interactions
    Jennifer E Kloepper
    Department of Dermatology, University of Lubeck, Lubeck, Germany
    J Invest Dermatol 135:679-89. 2015
    ..skin 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)...
  25. pmc Mitochondrial DNA stress primes the antiviral innate immune response
    A Phillip West
    Department of Pathology, Yale School of Medicine, New Haven, Connecticut 06520, USA
    Nature 520:553-7. 2015
    ..The abundant mtDNA-binding protein TFAM (transcription factor A, mitochondrial) regulates nucleoid architecture, abundance and segregation...
  26. pmc Mitochondrial transcription factor A regulation of mitochondrial degeneration in experimental diabetic neuropathy
    Krish Chandrasekaran
    Department of Neurology, University of Maryland, Baltimore, Maryland Veterans Affiars Maryland Health Care System
    Am J Physiol Endocrinol Metab 309:E132-41. 2015
    ..Mitochondrial transcription factor A (TFAM) wraps mtDNA and promotes mtDNA replication and transcription...
  27. pmc The regulation of mitochondrial transcription factor A (Tfam) expression during skeletal muscle cell differentiation
    Melania Collu-Marchese
    School of Kinesiology and Health Science, Muscle Health Research Centre, York University, Toronto, Ontario, Canada M3J 1P3
    Biosci Rep 35:. 2015
    ..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...
  28. pmc Cross-strand binding of TFAM to a single mtDNA molecule forms the mitochondrial nucleoid
    Christian Kukat
    Department of Mitochondrial Biology, Max Planck Institute for Biology of Ageing, 50931 Cologne, Germany FACS and Imaging Core Facility, Max Planck Institute for Biology of Ageing, 50931 Cologne, Germany
    Proc Natl Acad Sci U S A 112:11288-93. 2015
    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...
  29. pmc Cyclophilin D, a target for counteracting skeletal muscle dysfunction in mitochondrial myopathy
    Charlotte Gineste
    Department of Physiology and Pharmacology
    Hum Mol Genet 24:6580-7. 2015
    ..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 ..
  30. pmc Mitochondrial Transcription Factor A (TFAM) Binds to RNA Containing 4-Way Junctions and Mitochondrial tRNA
    Timothy A Brown
    Janelia Research Campus, Howard Hughes Medical Institute, Ashburn, Virginia, United States of America
    PLoS ONE 10:e0142436. 2015
    ..These structures are highly condensed by the DNA packaging protein, mitochondrial Transcription Factor A (TFAM)...
  31. pmc Dopaminergic Neurons Exhibit an Age-Dependent Decline in Electrophysiological Parameters in the MitoPark Mouse Model of Parkinson's Disease
    Sarah Y Branch
    Departments of Physiology
    J Neurosci 36:4026-37. 2016
    ..These findings may help inform future investigations into treatment targets for prodromal PD...
  32. pmc The Mitochondrial Respiratory Chain Is Required for Organismal Adaptation to Hypoxia
    Robert B Hamanaka
    Department of Medicine, The Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA
    Cell Rep 15:451-9. 2016
    ..Therefore, we conditionally deleted expression of TFAM in mouse epidermal keratinocytes...
  33. doi The mitochondrial electron transport chain is dispensable for proliferation and differentiation of epidermal progenitor cells
    Olivier R Baris
    Center for Physiology and Pathophysiology, Institute for Vegetative Physiology, University of Cologne, Cologne, Germany
    Stem Cells 29:1459-68. 2011
    ..This was achieved using a loxP-flanked mitochondrial transcription factor A (Tfam) allele in conjunction with a keratin 14 Cre transgene...
  34. pmc Intracellular and extracellular ATP coordinately regulate the inverse correlation between osteoclast survival and bone resorption
    Tsuyoshi Miyazaki
    Department of Geriatric Medicine, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Tokyo, Japan
    J Biol Chem 287:37808-23. 2012
    ..Severe ATP depletion, caused by disrupting mitochondrial transcription factor A (Tfam) gene, leads to increased bone-resorbing activity despite accelerated apoptosis...
  35. pmc Correlative 3D superresolution fluorescence and electron microscopy reveal the relationship of mitochondrial nucleoids to membranes
    Benjamin G Kopek
    Janelia Farm Research Campus, Howard Hughes Medical Institute, Ashburn, VA 20147, USA
    Proc Natl Acad Sci U S A 109:6136-41. 2012
    ....
  36. pmc TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication
    Dusanka Milenkovic
    Max Planck Institute for Biology of Ageing, Robert Koch Strasse 21, Cologne, Germany
    Hum Mol Genet 22:1983-93. 2013
    ..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...
  37. pmc Aberrant Schwann cell lipid metabolism linked to mitochondrial deficits leads to axon degeneration and neuropathy
    Andreu Viader
    Department of Genetics, Washington University School of Medicine, St Louis, MO 63110, USA
    Neuron 77:886-98. 2013
    ..Here, we investigate a mouse model of peripheral neuropathy secondary to SC mitochondrial dysfunction (Tfam-SCKOs)...
  38. doi Animal models of Parkinson's disease
    Fabio Blandini
    Interdepartmental Research Center for Parkinson s Disease, IRCCS National Neurological Institute C Mondino, Pavia, Italy
    FEBS J 279:1156-66. 2012
    ..Therefore, until the 'perfect' model is developed, indications to use one model or another will depend on the specific objectives that are being pursued...
  39. doi MicroRNA-494 regulates mitochondrial biogenesis in skeletal muscle through mitochondrial transcription factor A and Forkhead box j3
    Hirotaka Yamamoto
    Division of Endocrinology and Metabolism, Department of Medicine, Shiga University of Medical Science, Otsu, Japan
    Am J Physiol Endocrinol Metab 303:E1419-27. 2012
    ..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...
  40. pmc Lessons on conditional gene targeting in mouse adipose tissue
    Kevin Y Lee
    Section on Integrative Physiology and Metabolism, Joslin Diabetes Center, Harvard Medical School, Boston, Massachusetts, USA
    Diabetes 62:864-74. 2013
    ..Thus, different "adipocyte-specific" Cre lines display different degrees of efficiency and specificity, illustrating important differences that must be taken into account in their use for studying adipose biology...
  41. pmc Mitochondrial reactive oxygen species promote epidermal differentiation and hair follicle development
    Robert B Hamanaka
    Department of Medicine, The Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA
    Sci Signal 6:ra8. 2013
    ..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...
  42. pmc Mitochondrial genome instability and ROS enhance intestinal tumorigenesis in APC(Min/+) mice
    Dong Kyun Woo
    Department of Pathology, Yale University School of Medicine, New Haven, Connecticut 06520 8023, USA
    Am J Pathol 180:24-31. 2012
    ..Mitochondrial transcription factor A (TFAM) is required for expression and maintenance of mtDNA...
  43. pmc De novo neurogenesis in adult hypothalamus as a compensatory mechanism to regulate energy balance
    Andrew A Pierce
    Diabetes Center, University of California, San Francisco, San Francisco, California 94143, USA
    J Neurosci 30:723-30. 2010
    ..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...
  44. pmc Generation of Cre transgenic mice with postnatal RPE-specific ocular expression
    Jared Iacovelli
    F M Kirby Center for Molecular Ophthalmology, Scheie Eye Institute, University of Pennsylvania, Philadelphia, Pennsylvania, USA
    Invest Ophthalmol Vis Sci 52:1378-83. 2011
    ..To generate and characterize a constitutively active, RPE-specific, cre-expressing transgenic mouse line. This line can be used to create RPE-specific knockouts by crossing with mice harboring loxP-flanked (floxed) genes...
  45. ncbi Abnormal Ca(2+) release and catecholamine-induced arrhythmias in mitochondrial cardiomyopathy
    Pasi Tavi
    Department of Physiology and Pharmacology, Karolinska Institutet, SE 171 77 Stockholm, Sweden
    Hum Mol Genet 14:1069-76. 2005
    ..Here, we used mice with tissue-specific knockout of mitochondrial transcription factor A (Tfam) that leads to progressive cardiomyopathy...
  46. pmc A testis-specific gene encoding a nuclear high-mobility-group box protein located in elongating spermatids
    G Boissonneault
    Division of Cell and Developmental Genetics, University of California, San Francisco 94121
    Mol Cell Biol 13:4323-30. 1993
    ..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).
  47. ncbi A single mouse gene encodes the mitochondrial transcription factor A and a testis-specific nuclear HMG-box protein
    N G Larsson
    Department of Development Biology, Bechman Center for Molecular and Genetic Medicine, Stanford University School of Medicine, Calfornia 94305 5427, USA
    Nat Genet 13:296-302. 1996
    Mitochondrial transcription factor A (mtTFA) is a key regulator of mammalian mitochondrial DNA transcription...
  48. ncbi Structure and chromosomal localization of the mouse mitochondrial transcription factor A gene (Tfam)
    N G Larsson
    Department of Developmental Biology, Stanford University, School of Medicine, California 94305 5427, USA
    Mamm Genome 8:139-40. 1997
  49. ncbi The gene encoding guanidinoacetate methyltransferase (GAMT) maps to human chromosome 19 at band p13.3 and to mouse chromosome 10
    Y J Chae
    Laboratory of Biochemistry, Seoul National University College of Veterinary Medicine, Suwon, Republic of Korea
    Genomics 49:162-4. 1998
  50. ncbi Fine genetic and comparative mapping of the deafness mutation Ames waltzer on mouse chromosome 10
    E Zobeley
    Mental Health Research Institute, University of Michigan, Ann Arbor 48109 0720, USA
    Genomics 50:260-6. 1998
    ..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...
  51. ncbi Murine phospholipid hydroperoxide glutathione peroxidase: cDNA sequence, tissue expression, and mapping
    E A Knopp
    Division of Cardiology, Box 356422, University of Washington, 1959 N E Pacific St Seattle, Washington 98195, USA
    Mamm Genome 10:601-5. 1999
    ..This information may be valuable in characterizing the role of PHGPx in modulating susceptibility to lipid peroxide-mediated injury in inbred murine strains and for targeted disruption of the gene...
  52. pmc Genetic modification of survival in tissue-specific knockout mice with mitochondrial cardiomyopathy
    H Li
    Department of Molecular Medicine, CMM L8 02, Karolinska Hospital, S 17176 Stockholm, Sweden
    Proc Natl Acad Sci U S A 97:3467-72. 2000
    ..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 ..
  53. pmc Drosophila mitochondrial transcription factor A (d-TFAM) is dispensable for the transcription of mitochondrial DNA in Kc167 cells
    A Goto
    Graduate Course for Regulation of Biological Signals, Graduate School of Bioagricultural Sciences, Nagoya University, Furo cho, Chikusa ku, Nagoya shi 464 8601, Japan
    Biochem J 354:243-8. 2001
    We have cloned cDNA encoding Drosophila mitochondrial (mt) transcription factor A (d-TFAM)...
  54. ncbi Downregulation of Tfam and mtDNA copy number during mammalian spermatogenesis
    A Rantanen
    Department of Medical Nutrition, Karolinska Institutet, Novum, Huddinge Hospital, S 14186 Huddinge, 76 Stockholm, Sweden
    Mamm Genome 12:787-92. 2001
    Mitochondrial transcription factor A (Tfam) is required for mtDNA maintenance, and mitochondrial Tfam protein levels directly affect mtDNA copy number...
  55. ncbi Mitochondrial biogenesis and thyroid status maturation in brown fat require CCAAT/enhancer-binding protein alpha
    M Carmen Carmona
    Departament de Bioquimica i Biologia Molecular, Universitat de Barcelona, Barcelona E 08028, Spain
    J Biol Chem 277:21489-98. 2002
    ....
  56. ncbi Upregulation of VEGF-A without angiogenesis in a mouse model of dilated cardiomyopathy caused by mitochondrial dysfunction
    Emma Tham
    Department of Molecular Medicine, Karolinska Institutet, Stockholm, Sweden
    J Histochem Cytochem 50:935-44. 2002
    ..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...
  57. pmc A switch in metabolism precedes increased mitochondrial biogenesis in respiratory chain-deficient mouse hearts
    Anna Hansson
    Department of Medical Nutrition and Biosciences, Karolinska Institutet, Novum, Karolinska University Hospital, S 141 86 Stockholm, Sweden
    Proc Natl Acad Sci U S A 101:3136-41. 2004
    ..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 ..
  58. pmc The mitochondrial RNA polymerase contributes critically to promoter specificity in mammalian cells
    Martina Gaspari
    Department of Medical Nutrition, Karolinska Institute, Novum, Karolinska University Hospital, Stockholm, Sweden
    EMBO J 23:4606-14. 2004
    ..depends on three proteins: mitochondrial RNA polymerase (POLRMT), mitochondrial transcription factor A (TFAM) and mitochondrial transcription factor B2 (TFB2M)...
  59. pmc Mitochondrial biogenesis by NO yields functionally active mitochondria in mammals
    Enzo Nisoli
    Department of Preclinical Sciences, Laboratorio Interdisciplinare Technologie Avanzate LITA Vialba, University of Milan, 20157 Milan, Italy
    Proc Natl Acad Sci U S A 101:16507-12. 2004
    ..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...
  60. pmc mTOR-mediated dedifferentiation of the retinal pigment epithelium initiates photoreceptor degeneration in mice
    Chen Zhao
    Department of Genetics, Stanford University School of Medicine, Stanford, California 94305 5120, USA
    J Clin Invest 121:369-83. 2011
    ..Our findings provide a rationale for mTOR pathway inhibition as a therapeutic strategy for retinal degenerative diseases involving RPE stress...
  61. ncbi Molecular control of mitochondrial function in preimplantation mouse embryos
    Jacob Thundathil
    Centre de Recherche en Reproduction Animale, Faculte de Medecine Veterinaire, Universite de Montreal, Quebec, Canada
    Mol Reprod Dev 71:405-13. 2005
    ....
  62. ncbi Respiratory chain dysfunction in skeletal muscle does not cause insulin resistance
    Anna Wredenberg
    Department of Laboratory Medicine, Karolinska Insitutet, Karolinska University Hospital, Stockholm, Sweden
    Biochem Biophys Res Commun 350:202-7. 2006
    ..In summary, our findings indicate that mitochondrial dysfunction in skeletal muscle is not a primary etiological event in DM2...
  63. ncbi Proteolytic processing of OPA1 links mitochondrial dysfunction to alterations in mitochondrial morphology
    Stéphane Duvezin-Caubet
    Adolf Butenandt Institut für Physiologische Chemie, Ludwig Maximilians Universitat Munchen, Butenandtstrasse 5, 81377 Munchen, Germany
    J Biol Chem 281:37972-9. 2006
    ..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 ..
  64. ncbi Essential role of mitochondrial function in adiponectin synthesis in adipocytes
    Eun Hee Koh
    Department of Internal Medicine, University of Ulsan College of Medicine, Poongnap dong, Songpa Ku, Seoul 138 736, Korea
    Diabetes 56:2973-81. 2007
    ..This study was undertaken to test the hypothesis that mitochondrial function is linked to adiponectin synthesis in adipocytes...
  65. doi Lrrk2 and alpha-synuclein are co-regulated in rodent striatum
    Marie Westerlund
    Department of Neuroscience, Karolinska Institutet, Retzius väg 8 B2 4, S 17177 Stockholm, Sweden
    Mol Cell Neurosci 39:586-91. 2008
    ..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...
  66. doi Increased mitochondrial Ca2+ and decreased sarcoplasmic reticulum Ca2+ in mitochondrial myopathy
    Jan Aydin
    Present address Max Planck Institute for Biology of Ageing, Gleueler Str 50a, Cologne, Germany
    Hum Mol Genet 18:278-88. 2009
    ..we used mice with skeletal-muscle-specific disruption of the nuclear gene for mitochondrial transcription factor A (Tfam) to study whether changes in cellular Ca(2+) handling is part of the mechanism of muscle dysfunction in ..
  67. pmc Functional requirement of AgRP and NPY neurons in ovarian cycle-dependent regulation of food intake
    Louise E Olofsson
    Diabetes Center, University of California, San Francisco, CA 94143, USA
    Proc Natl Acad Sci U S A 106:15932-7. 2009
    ..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...
  68. pmc Functional role of c-Jun-N-terminal kinase in feeding regulation
    Elizabeth K Unger
    UCSF Diabetes Center, Department of Anatomy, University of California, San Francisco, California 94143, USA
    Endocrinology 151:671-82. 2010
    ..Thus, JNK may integrate diverse metabolic signals and differentially regulate feeding under distinct physiological conditions...
  69. doi Overexpression of mitochondrial transcription factor A (TFAM) ameliorates delayed neuronal death due to transient forebrain ischemia in mice
    Masaaki Hokari
    Department of Neurosurgery and Cardiovascular Medicine, Hokkaido University Graduate School of Medicine, Sapporo, Japan
    Neuropathology 30:401-7. 2010
    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...
  70. pmc The role of mitochondrial DNA copy number in mammalian fertility
    Timothy Wai
    Montreal Neurological Institute and Department of Human Genetics, McGill University, Montreal, Quebec, Canada
    Biol Reprod 83:52-62. 2010
    ..mtDNA copy number on fertility, we genetically manipulated mtDNA copy number in the mouse by deleting one copy of Tfam, an essential component of the mitochondrial nucleoid, at different stages of germline development...
  71. pmc Mitochondrial metabolism and ROS generation are essential for Kras-mediated tumorigenicity
    Frank Weinberg
    Division of Pulmonary and Critical Care, Department of Medicine, Robert H Lurie Comprehensive Cancer Center, Northwestern University Medical School Chicago, IL 60611, USA
    Proc Natl Acad Sci U S A 107:8788-93. 2010
    ..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...
  72. pmc Rb regulates fate choice and lineage commitment in vivo
    Eliezer Calo
    David H Koch Institute for Integrative Cancer Research at MIT, Massachusetts Institute of Technology, 77 Massachusetts Avenue, Cambridge, Massachusetts 02139, USA
    Nature 466:1110-4. 2010
    ..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...