B6N.129-Tfam^tm1Lrsn/Kctt

Status	Available to order
EMMA ID	EM:13208
Citation information	RRID:IMSR_EM:13208 Research Resource Identifiers (RRID) are persistent unique ID numbers assigned to help researchers cite key resources (e.g. antibodies, model organisms and software projects) in the biomedical literature to improve transparency and reproducibility in research. See https://www.rrids.org/ for more information.
International strain name	B6N.129-Tfam^tm1Lrsn/Kctt
Alternative name	Tfam loxP
Strain type	Targeted Mutant Strains : Conditional mutation
Allele/Transgene symbol	Tfam^tm1Lrsn
Gene/Transgene symbol	Tfam

Information from provider

Provider	Nils-Göran Larsson
Provider affiliation	Karolinska Institutet
Genetic information	The mitochondrial transcription factor A (Tfam) locus was altered in the following way: a loxP site was introduced upstream of exon 6 and downstream of exon 7 to allow later excision of these two exons. A PGK-neo cassette was introduced to allow selection of targeted ES-cell clones in the production of this mouse. When bred to mice that express cre recombinase, resulting offspring will have exons 6-7 and the neo cassette deleted in cre-expressing tissues. The generation of this mutant has been described in Larsson et al., 1998. MitoPark mice can be generated by crossing this line (EM:13208, Tfam loxP) to the Dat-cre line (EM:13209). See Ekstrand et al., 2007 for further details.
Phenotypic information	Homozygous: Mice that are homozygous for this allele are viable and fertile. Heterozygous: Viable and fertile.
Breeding history	Males used for sperm freezing are on C57BL/6N background (at least 10 generations of backcrossing).
References	Abnormal Ca(2+) release and catecholamine-induced arrhythmias in mitochondrial cardiomyopathy.;Tavi Pasi, Hansson Anna, Zhang Shi-Jin, Larsson Nils-Göran, Westerblad Håkan, ;2005;Human molecular genetics;14;1069-76; 15757973 Late-onset corticohippocampal neurodepletion attributable to catastrophic failure of oxidative phosphorylation in MILON mice.;Sörensen L, Ekstrand M, Silva J P, Lindqvist E, Xu B, Rustin P, Olson L, Larsson N G, ;2001;The Journal of neuroscience : the official journal of the Society for Neuroscience;21;8082-90; 11588181 Maintenance of respiratory chain function in mouse hearts with severely impaired mtDNA transcription.;Freyer Christoph, Park Chan Bae, Ekstrand Mats I, Shi Yonghong, Khvorostova Julia, Wibom Rolf, Falkenberg Maria, Gustafsson Claes M, Larsson Nils-Göran, ;2010;Nucleic acids research;38;6577-88; 20566479 Increased mitochondrial Ca2+ and decreased sarcoplasmic reticulum Ca2+ in mitochondrial myopathy.;Aydin Jan, Andersson Daniel C, Hänninen Sandra L, Wredenberg Anna, Tavi Pasi, Park Chan Bae, Larsson Nils-Göran, Bruton Joseph D, Westerblad Håkan, ;2009;Human molecular genetics;18;278-88; 18945718 Mitochondrial Dysfunction Combined with High Calcium Load Leads to Impaired Antioxidant Defense Underlying the Selective Loss of Nigral Dopaminergic Neurons.;Ricke Konrad M, Paß Thomas, Kimoloi Sammy, Fährmann Kai, Jüngst Christian, Schauss Astrid, Baris Olivier R, Aradjanski Marijana, Trifunovic Aleksandra, Eriksson Faelker Therese M, Bergami Matteo, Wiesner Rudolf J, ;2020;The Journal of neuroscience : the official journal of the Society for Neuroscience;40;1975-1986; 32005765 Elevated COUP-TFII expression in dopaminergic neurons accelerates the progression of Parkinson's disease through mitochondrial dysfunction.;Kao Chung-Yang, Xu Mafei, Wang Leiming, Lin Shih-Chieh, Lee Hui-Ju, Duraine Lita, Bellen Hugo J, Goldstein David S, Tsai Sophia Y, Tsai Ming-Jer, ;2020;PLoS genetics;16;e1008868; 32579581 Adipose-specific deletion of TFAM increases mitochondrial oxidation and protects mice against obesity and insulin resistance.;Vernochet Cecile, Mourier Arnaud, Bezy Olivier, Macotela Yazmin, Boucher Jeremie, Rardin Matthew J, An Ding, Lee Kevin Y, Ilkayeva Olga R, Zingaretti Cristina M, Emanuelli Brice, Smyth Graham, Cinti Saverio, Newgard Christopher B, Gibson Bradford W, Larsson Nils-Göran, Kahn C Ronald, ;2012;Cell metabolism;16;765-76; 23168219 MitoPark transgenic mouse model recapitulates the gastrointestinal dysfunction and gut-microbiome changes of Parkinson's disease.;Ghaisas Shivani, Langley Monica R, Palanisamy Bharathi N, Dutta Somak, Narayanaswamy Kirthi, Plummer Paul J, Sarkar Souvarish, Ay Muhammet, Jin Huajun, Anantharam Vellareddy, Kanthasamy Arthi, Kanthasamy Anumantha G, ;2019;Neurotoxicology;75;186-199; 31505196 Cerebrospinal fluid neurofilament light chain as a biomarker of neurodegeneration in the Tg4510 and MitoPark mouse models.;Clement Amalie, Mitchelmore Cathy, Andersson Daniel R, Asuni Ayodeji A, ;2017;Neuroscience;354;101-109; 28461218 Non-toxic HSC Transplantation-Based Macrophage/Microglia-Mediated GDNF Delivery for Parkinson's Disease.;Chen Cang, Guderyon Michael J, Li Yang, Ge Guo, Bhattacharjee Anindita, Ballard Cori, He Zhixu, Masliah Eliezer, Clark Robert A, O'Connor Jason C, Li Senlin, ;2020;Molecular therapy. Methods & clinical development;17;83-98; 31890743 Chronic A2A antagonist treatment alleviates parkinsonian locomotor deficiency in MitoPark mice.;Marcellino Daniel, Lindqvist Eva, Schneider Marion, Müller Christa E, Fuxe Kjell, Olson Lars, Galter Dagmar, ;2010;Neurobiology of disease;40;460-6; 20656029 Progressively disrupted somatodendritic morphology in dopamine neurons in a mouse Parkinson's model.;Lynch William B, Tschumi Christopher W, Sharpe Amanda L, Branch Sarah Y, Chen Cang, Ge Guo, Li Senlin, Beckstead Michael J, ;2018;Movement disorders : official journal of the Movement Disorder Society;33;1928-1937; 30440089 MitoPark mice, an animal model of Parkinson's disease, show enhanced prepulse inhibition of acoustic startle and no loss of gating in response to the adenosine A(2A) antagonist SCH 412348.;Grauer Steven M, Hodgson Robert, Hyde Lynn A, ;2014;Psychopharmacology;231;1325-37; 24150248 Age-associated mosaic respiratory chain deficiency causes trans-neuronal degeneration.;Dufour Eric, Terzioglu Mügen, Sterky Fredrik Hansson, Sörensen Lene, Galter Dagmar, Olson Lars, Wilbertz Johannes, Larsson Nils-Göran, ;2008;Human molecular genetics;17;1418-26; 18245781 A potent and selective metabotropic glutamate receptor 4 positive allosteric modulator improves movement in rodent models of Parkinson's disease.;Le Poul Emmanuel, Boléa Christelle, Girard Francoise, Poli Sonia, Charvin Delphine, Campo Brice, Bortoli Julien, Bessif Abdhelak, Luo Bin, Koser Amy Jo, Hodge Lisa M, Smith Karen M, DiLella Anthony G, Liverton Nigel, Hess Fred, Browne Susan E, Reynolds Ian J, ;2012;The Journal of pharmacology and experimental therapeutics;343;167-77; 22787118 Lrrk2 and alpha-synuclein are co-regulated in rodent striatum.;Westerlund Marie, Ran Caroline, Borgkvist Anders, Sterky Fredrik H, Lindqvist Eva, Lundströmer Karin, Pernold Karin, Brené Stefan, Kallunki Pekka, Fisone Gilberto, Olson Lars, Galter Dagmar, ;2008;Molecular and cellular neurosciences;39;586-91; 18790059 Impaired nigrostriatal function precedes behavioral deficits in a genetic mitochondrial model of Parkinson's disease.;Good Cameron H, Hoffman Alexander F, Hoffer Barry J, Chefer Vladimir I, Shippenberg Toni S, Bäckman Cristina M, Larsson Nils-Göran, Olson Lars, Gellhaar Sandra, Galter Dagmar, Lupica Carl R, ;2011;FASEB journal : official publication of the Federation of American Societies for Experimental Biology;25;1333-44; 21233488 The mitochondrial electron transport chain is dispensable for proliferation and differentiation of epidermal progenitor cells.;Baris Olivier R, Klose Anke, Kloepper Jennifer E, Weiland Daniela, Neuhaus Johannes F G, Schauen Matthias, Wille Anna, Müller Alexander, Merkwirth Carsten, Langer Thomas, Larsson Nils-Göran, Krieg Thomas, Tobin Desmond J, Paus Ralf, Wiesner Rudolf J, ;2011;Stem cells (Dayton, Ohio);29;1459-68; 21780252 Progressive parkinsonism in mice with respiratory-chain-deficient dopamine neurons.;Ekstrand Mats I, Terzioglu Mügen, Galter Dagmar, Zhu Shunwei, Hofstetter Christoph, Lindqvist Eva, Thams Sebastian, Bergstrand Anita, Hansson Fredrik Sterky, Trifunovic Aleksandra, Hoffer Barry, Cullheim Staffan, Mohammed Abdul H, Olson Lars, Larsson Nils-Göran, ;2007;Proceedings of the National Academy of Sciences of the United States of America;104;1325-30; 17227870 Modulation of mtDNA copy number ameliorates the pathological consequences of a heteroplasmic mtDNA mutation in the mouse.;Filograna R, Koolmeister C, Upadhyay M, Pajak A, Clemente P, Wibom R, Simard M L, Wredenberg A, Freyer C, Stewart J B, Larsson N G, ;2019;Science advances;5;eaav9824; 30949583 Impaired insulin secretion and beta-cell loss in tissue-specific knockout mice with mitochondrial diabetes.;Silva J P, Köhler M, Graff C, Oldfors A, Magnuson M A, Berggren P O, Larsson N G, ;2000;Nature genetics;26;336-40; 11062475 MitoPark mice mirror the slow progression of key symptoms and L-DOPA response in Parkinson's disease.;Galter D, Pernold K, Yoshitake T, Lindqvist E, Hoffer B, Kehr J, Larsson N-G, Olson L, ;2010;Genes, brain, and behavior;9;173-81; 20002202 Chronic L-DOPA induces hyperactivity, normalization of gait and dyskinetic behavior in MitoPark mice.;Gellhaar S, Marcellino D, Abrams M B, Galter D, ;2015;Genes, brain, and behavior;14;260-70; 25752644 MLi-2, a Potent, Selective, and Centrally Active Compound for Exploring the Therapeutic Potential and Safety of LRRK2 Kinase Inhibition.;Fell Matthew J, Mirescu Christian, Basu Kallol, Cheewatrakoolpong Boonlert, DeMong Duane E, Ellis J Michael, Hyde Lynn A, Lin Yinghui, Markgraf Carrie G, Mei Hong, Miller Michael, Poulet Frederique M, Scott Jack D, Smith Michelle D, Yin Zhizhang, Zhou Xiaoping, Parker Eric M, Kennedy Matthew E, Morrow John A, ;2015;The Journal of pharmacology and experimental therapeutics;355;397-409; 26407721 GDNF-expressing macrophages mitigate loss of dopamine neurons and improve Parkinsonian symptoms in MitoPark mice.;Chen Cang, Li Xiuhua, Ge Guo, Liu Jingwei, Biju K C, Laing Suzette D, Qian Yusheng, Ballard Cori, He Zhixu, Masliah Eliezer, Clark Robert A, O'Connor Jason C, Li Senlin, ;2018;Scientific reports;8;5460; 29615705 Adipose tissue mitochondrial dysfunction triggers a lipodystrophic syndrome with insulin resistance, hepatosteatosis, and cardiovascular complications.;Vernochet Cecile, Damilano Federico, Mourier Arnaud, Bezy Olivier, Mori Marcelo A, Smyth Graham, Rosenzweig Anthony, Larsson Nils-Göran, Kahn C Ronald, ;2014;FASEB journal : official publication of the Federation of American Societies for Experimental Biology;28;4408-19; 25005176 Effects of the selective adenosine A2A receptor antagonist, SCH 412348, on the parkinsonian phenotype of MitoPark mice.;Smith Karen M, Browne Susan E, Jayaraman Srinivasan, Bleickardt Carina J, Hodge Lisa M, Lis Edward, Yao Leon, Rittle Sunday L, Innocent Nathalie, Mullins Deborra E, Boykow George, Reynolds Ian J, Hill David, Parker Eric M, Hodgson Robert A, ;2014;European journal of pharmacology;728;31-8; 24486705 Loss of dopamine disrupts circadian rhythms in a mouse model of Parkinson's disease.;Fifel Karim, Cooper Howard M, ;2014;Neurobiology of disease;71;359-69; 25171792 Molecular mechanisms underlying protective effects of quercetin against mitochondrial dysfunction and progressive dopaminergic neurodegeneration in cell culture and MitoPark transgenic mouse models of Parkinson's Disease.;Ay Muhammet, Luo Jie, Langley Monica, Jin Huajun, Anantharam Vellareddy, Kanthasamy Arthi, Kanthasamy Anumantha G, ;2017;Journal of neurochemistry;141;766-782; 28376279 A switch in metabolism precedes increased mitochondrial biogenesis in respiratory chain-deficient mouse hearts.;Hansson Anna, Hance Nicole, Dufour Eric, Rantanen Anja, Hultenby Kjell, Clayton David A, Wibom Rolf, Larsson Nils-Göran, ;2004;Proceedings of the National Academy of Sciences of the United States of America;101;3136-41; 14978272 Cognitive dysfunction precedes the onset of motor symptoms in the MitoPark mouse model of Parkinson's disease.;Li Xiuhua, Redus Laney, Chen Cang, Martinez Paul A, Strong Randy, Li Senlin, O'Connor Jason C, ;2013;PloS one;8;e71341; 23977020 Release parameters during progressive degeneration of dopamine neurons in a mouse model reveal earlier impairment of spontaneous than forced behaviors.;Chen Yuan-Hao, Hsieh Tsung-Hsun, Kuo Tung-Tai, Kao Jen-Hsin, Ma Kuo-Hsing, Huang Eagle Yi-Kung, Chou Yu-Ching, Olson Lars, Hoffer Barry J, ;2019;Journal of neurochemistry;150;56-73; 30933310 Mito-Apocynin Prevents Mitochondrial Dysfunction, Microglial Activation, Oxidative Damage, and Progressive Neurodegeneration in MitoPark Transgenic Mice.;Langley Monica, Ghosh Anamitra, Charli Adhithiya, Sarkar Souvarish, Ay Muhammet, Luo Jie, Zielonka Jacek, Brenza Timothy, Bennett Brian, Jin Huajun, Ghaisas Shivani, Schlichtmann Benjamin, Kim Dongsuk, Anantharam Vellareddy, Kanthasamy Arthi, Narasimhan Balaji, Kalyanaraman Balaraman, Kanthasamy Anumantha G, ;2017;Antioxidants & redox signaling;27;1048-1066; 28375739 Altered glutamate release in the dorsal striatum of the MitoPark mouse model of Parkinson's disease.;Farrand Ariana Q, Gregory Rebecca A, Bäckman Cristina M, Helke Kristi L, Boger Heather A, ;2016;Brain research;1651;88-94; 27659966 Dopaminergic Neurons Exhibit an Age-Dependent Decline in Electrophysiological Parameters in the MitoPark Mouse Model of Parkinson's Disease.;Branch Sarah Y, Chen Cang, Sharma Ramaswamy, Lechleiter James D, Li Senlin, Beckstead Michael J, ;2016;The Journal of neuroscience : the official journal of the Society for Neuroscience;36;4026-37; 27053209 PTEN deletion enhances survival, neurite outgrowth and function of dopamine neuron grafts to MitoPark mice.;Zhang YaJun, Granholm Ann-Charlotte, Huh Kyounghee, Shan Lufei, Diaz-Ruiz Oscar, Malik Nasir, Olson Lars, Hoffer Barry J, Lupica Carl R, Hoffman Alexander F, Bäckman Cristina M, ;2012;Brain : a journal of neurology;135;2736-49; 22961549 Delayed Dopamine Dysfunction and Motor Deficits in Female Parkinson Model Mice.;Chen Yuan-Hao, Wang Vicki, Huang Eagle Yi-Kung, Chou Yu-Ching, Kuo Tung-Tai, Olson Lars, Hoffer Barry J, ;2019;International journal of molecular sciences;20;261-9; 31835787 L-Dopa induced dyskinesias in Parkinsonian mice: Disease severity or L-Dopa history.;Shan Lufei, Diaz Oscar, Zhang Yajun, Ladenheim Bruce, Cadet Jean-Lud, Chiang Yung-Hsiao, Olson Lars, Hoffer Barry J, Bäckman Cristina M, ;2015;Brain research;1618;106-10; 26086365 Manipulation of mitochondrial DNA gene expression in the mouse.;Silva José P, Larsson Nils-Göran, ;2002;Biochimica et biophysica acta;1555;146301; 12206900 Voluntary exercise delays progressive deterioration of markers of metabolism and behavior in a mouse model of Parkinson's disease.;Lai Jing-Huei, Chen Kai-Yun, Wu John Chung-Che, Olson Lars, Brené Stefan, Huang Chi-Zong, Chen Yen-Hua, Kang Shuo-Jhen, Ma Kuo-Hsing, Hoffer Barry J, Hsieh Tsung-Hsun, Chiang Yung-Hsiao, ;2019;Brain research;1720;429-436.e4; 31226324 Increased Total mtDNA Copy Number Cures Male Infertility Despite Unaltered mtDNA Mutation Load.;Jiang Min, Kauppila Timo Eino Sakari, Motori Elisa, Li Xinping, Atanassov Ilian, Folz-Donahue Kat, Bonekamp Nina Anna, Albarran-Gutierrez Sara, Stewart James Bruce, Larsson Nils-Göran, ;2017;Cell metabolism;26;15066-71; 28768180 Increased mitochondrial mass in mitochondrial myopathy mice.;Wredenberg Anna, Wibom Rolf, Wilhelmsson Hans, Graff Caroline, Wiener Heidi H, Burden Steven J, Oldfors Anders, Westerblad Håkan, Larsson Nils-Göran, ;2002;Proceedings of the National Academy of Sciences of the United States of America;99;231-6; 12417746 Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice.;Larsson N G, Wang J, Wilhelmsson H, Oldfors A, Rustin P, Lewandoski M, Barsh G S, Clayton D A, ;1998;Nature genetics;18;12937-42; 9500544 Impaired mitochondrial transport and Parkin-independent degeneration of respiratory chain-deficient dopamine neurons in vivo.;Sterky Fredrik H, Lee Seungmin, Wibom Rolf, Olson Lars, Larsson Nils-Göran, ;2011;Proceedings of the National Academy of Sciences of the United States of America;108;e0151884; 21768369 Multimodal MRI Evaluation of the MitoPark Mouse Model of Parkinson's Disease.;Cong Linlin, Muir Eric R, Chen Cang, Qian Yusheng, Liu Jingwei, Biju K C, Clark Robert A, Li Senlin, Duong Timothy Q, ;2016;PloS one;11;; 27003179
Homozygous fertile	yes
Homozygous viable	yes
Homozygous matings required	no
Immunocompromised	no

Information from EMMA

Archiving centre	Karolinska Institutet, Stockholm, Sweden
Animals used for archiving	homozygous C57BL/6N males

Literature references

Abnormal Ca(2+) release and catecholamine-induced arrhythmias in mitochondrial cardiomyopathy.;Tavi Pasi, Hansson Anna, Zhang Shi-Jin, Larsson Nils-Göran, Westerblad Håkan, ;2005;Human molecular genetics;14;1069-76; 15757973
Late-onset corticohippocampal neurodepletion attributable to catastrophic failure of oxidative phosphorylation in MILON mice.;Sörensen L, Ekstrand M, Silva J P, Lindqvist E, Xu B, Rustin P, Olson L, Larsson N G, ;2001;The Journal of neuroscience : the official journal of the Society for Neuroscience;21;8082-90; 11588181
Maintenance of respiratory chain function in mouse hearts with severely impaired mtDNA transcription.;Freyer Christoph, Park Chan Bae, Ekstrand Mats I, Shi Yonghong, Khvorostova Julia, Wibom Rolf, Falkenberg Maria, Gustafsson Claes M, Larsson Nils-Göran, ;2010;Nucleic acids research;38;6577-88; 20566479
Increased mitochondrial Ca2+ and decreased sarcoplasmic reticulum Ca2+ in mitochondrial myopathy.;Aydin Jan, Andersson Daniel C, Hänninen Sandra L, Wredenberg Anna, Tavi Pasi, Park Chan Bae, Larsson Nils-Göran, Bruton Joseph D, Westerblad Håkan, ;2009;Human molecular genetics;18;278-88; 18945718
Mitochondrial Dysfunction Combined with High Calcium Load Leads to Impaired Antioxidant Defense Underlying the Selective Loss of Nigral Dopaminergic Neurons.;Ricke Konrad M, Paß Thomas, Kimoloi Sammy, Fährmann Kai, Jüngst Christian, Schauss Astrid, Baris Olivier R, Aradjanski Marijana, Trifunovic Aleksandra, Eriksson Faelker Therese M, Bergami Matteo, Wiesner Rudolf J, ;2020;The Journal of neuroscience : the official journal of the Society for Neuroscience;40;1975-1986; 32005765
Elevated COUP-TFII expression in dopaminergic neurons accelerates the progression of Parkinson's disease through mitochondrial dysfunction.;Kao Chung-Yang, Xu Mafei, Wang Leiming, Lin Shih-Chieh, Lee Hui-Ju, Duraine Lita, Bellen Hugo J, Goldstein David S, Tsai Sophia Y, Tsai Ming-Jer, ;2020;PLoS genetics;16;e1008868; 32579581
Adipose-specific deletion of TFAM increases mitochondrial oxidation and protects mice against obesity and insulin resistance.;Vernochet Cecile, Mourier Arnaud, Bezy Olivier, Macotela Yazmin, Boucher Jeremie, Rardin Matthew J, An Ding, Lee Kevin Y, Ilkayeva Olga R, Zingaretti Cristina M, Emanuelli Brice, Smyth Graham, Cinti Saverio, Newgard Christopher B, Gibson Bradford W, Larsson Nils-Göran, Kahn C Ronald, ;2012;Cell metabolism;16;765-76; 23168219
MitoPark transgenic mouse model recapitulates the gastrointestinal dysfunction and gut-microbiome changes of Parkinson's disease.;Ghaisas Shivani, Langley Monica R, Palanisamy Bharathi N, Dutta Somak, Narayanaswamy Kirthi, Plummer Paul J, Sarkar Souvarish, Ay Muhammet, Jin Huajun, Anantharam Vellareddy, Kanthasamy Arthi, Kanthasamy Anumantha G, ;2019;Neurotoxicology;75;186-199; 31505196
Cerebrospinal fluid neurofilament light chain as a biomarker of neurodegeneration in the Tg4510 and MitoPark mouse models.;Clement Amalie, Mitchelmore Cathy, Andersson Daniel R, Asuni Ayodeji A, ;2017;Neuroscience;354;101-109; 28461218
Non-toxic HSC Transplantation-Based Macrophage/Microglia-Mediated GDNF Delivery for Parkinson's Disease.;Chen Cang, Guderyon Michael J, Li Yang, Ge Guo, Bhattacharjee Anindita, Ballard Cori, He Zhixu, Masliah Eliezer, Clark Robert A, O'Connor Jason C, Li Senlin, ;2020;Molecular therapy. Methods & clinical development;17;83-98; 31890743
Chronic A2A antagonist treatment alleviates parkinsonian locomotor deficiency in MitoPark mice.;Marcellino Daniel, Lindqvist Eva, Schneider Marion, Müller Christa E, Fuxe Kjell, Olson Lars, Galter Dagmar, ;2010;Neurobiology of disease;40;460-6; 20656029
Progressively disrupted somatodendritic morphology in dopamine neurons in a mouse Parkinson's model.;Lynch William B, Tschumi Christopher W, Sharpe Amanda L, Branch Sarah Y, Chen Cang, Ge Guo, Li Senlin, Beckstead Michael J, ;2018;Movement disorders : official journal of the Movement Disorder Society;33;1928-1937; 30440089
MitoPark mice, an animal model of Parkinson's disease, show enhanced prepulse inhibition of acoustic startle and no loss of gating in response to the adenosine A(2A) antagonist SCH 412348.;Grauer Steven M, Hodgson Robert, Hyde Lynn A, ;2014;Psychopharmacology;231;1325-37; 24150248
Age-associated mosaic respiratory chain deficiency causes trans-neuronal degeneration.;Dufour Eric, Terzioglu Mügen, Sterky Fredrik Hansson, Sörensen Lene, Galter Dagmar, Olson Lars, Wilbertz Johannes, Larsson Nils-Göran, ;2008;Human molecular genetics;17;1418-26; 18245781
A potent and selective metabotropic glutamate receptor 4 positive allosteric modulator improves movement in rodent models of Parkinson's disease.;Le Poul Emmanuel, Boléa Christelle, Girard Francoise, Poli Sonia, Charvin Delphine, Campo Brice, Bortoli Julien, Bessif Abdhelak, Luo Bin, Koser Amy Jo, Hodge Lisa M, Smith Karen M, DiLella Anthony G, Liverton Nigel, Hess Fred, Browne Susan E, Reynolds Ian J, ;2012;The Journal of pharmacology and experimental therapeutics;343;167-77; 22787118
Lrrk2 and alpha-synuclein are co-regulated in rodent striatum.;Westerlund Marie, Ran Caroline, Borgkvist Anders, Sterky Fredrik H, Lindqvist Eva, Lundströmer Karin, Pernold Karin, Brené Stefan, Kallunki Pekka, Fisone Gilberto, Olson Lars, Galter Dagmar, ;2008;Molecular and cellular neurosciences;39;586-91; 18790059
Impaired nigrostriatal function precedes behavioral deficits in a genetic mitochondrial model of Parkinson's disease.;Good Cameron H, Hoffman Alexander F, Hoffer Barry J, Chefer Vladimir I, Shippenberg Toni S, Bäckman Cristina M, Larsson Nils-Göran, Olson Lars, Gellhaar Sandra, Galter Dagmar, Lupica Carl R, ;2011;FASEB journal : official publication of the Federation of American Societies for Experimental Biology;25;1333-44; 21233488
The mitochondrial electron transport chain is dispensable for proliferation and differentiation of epidermal progenitor cells.;Baris Olivier R, Klose Anke, Kloepper Jennifer E, Weiland Daniela, Neuhaus Johannes F G, Schauen Matthias, Wille Anna, Müller Alexander, Merkwirth Carsten, Langer Thomas, Larsson Nils-Göran, Krieg Thomas, Tobin Desmond J, Paus Ralf, Wiesner Rudolf J, ;2011;Stem cells (Dayton, Ohio);29;1459-68; 21780252
Progressive parkinsonism in mice with respiratory-chain-deficient dopamine neurons.;Ekstrand Mats I, Terzioglu Mügen, Galter Dagmar, Zhu Shunwei, Hofstetter Christoph, Lindqvist Eva, Thams Sebastian, Bergstrand Anita, Hansson Fredrik Sterky, Trifunovic Aleksandra, Hoffer Barry, Cullheim Staffan, Mohammed Abdul H, Olson Lars, Larsson Nils-Göran, ;2007;Proceedings of the National Academy of Sciences of the United States of America;104;1325-30; 17227870
Modulation of mtDNA copy number ameliorates the pathological consequences of a heteroplasmic mtDNA mutation in the mouse.;Filograna R, Koolmeister C, Upadhyay M, Pajak A, Clemente P, Wibom R, Simard M L, Wredenberg A, Freyer C, Stewart J B, Larsson N G, ;2019;Science advances;5;eaav9824; 30949583
Impaired insulin secretion and beta-cell loss in tissue-specific knockout mice with mitochondrial diabetes.;Silva J P, Köhler M, Graff C, Oldfors A, Magnuson M A, Berggren P O, Larsson N G, ;2000;Nature genetics;26;336-40; 11062475
MitoPark mice mirror the slow progression of key symptoms and L-DOPA response in Parkinson's disease.;Galter D, Pernold K, Yoshitake T, Lindqvist E, Hoffer B, Kehr J, Larsson N-G, Olson L, ;2010;Genes, brain, and behavior;9;173-81; 20002202
Chronic L-DOPA induces hyperactivity, normalization of gait and dyskinetic behavior in MitoPark mice.;Gellhaar S, Marcellino D, Abrams M B, Galter D, ;2015;Genes, brain, and behavior;14;260-70; 25752644
MLi-2, a Potent, Selective, and Centrally Active Compound for Exploring the Therapeutic Potential and Safety of LRRK2 Kinase Inhibition.;Fell Matthew J, Mirescu Christian, Basu Kallol, Cheewatrakoolpong Boonlert, DeMong Duane E, Ellis J Michael, Hyde Lynn A, Lin Yinghui, Markgraf Carrie G, Mei Hong, Miller Michael, Poulet Frederique M, Scott Jack D, Smith Michelle D, Yin Zhizhang, Zhou Xiaoping, Parker Eric M, Kennedy Matthew E, Morrow John A, ;2015;The Journal of pharmacology and experimental therapeutics;355;397-409; 26407721
GDNF-expressing macrophages mitigate loss of dopamine neurons and improve Parkinsonian symptoms in MitoPark mice.;Chen Cang, Li Xiuhua, Ge Guo, Liu Jingwei, Biju K C, Laing Suzette D, Qian Yusheng, Ballard Cori, He Zhixu, Masliah Eliezer, Clark Robert A, O'Connor Jason C, Li Senlin, ;2018;Scientific reports;8;5460; 29615705
Adipose tissue mitochondrial dysfunction triggers a lipodystrophic syndrome with insulin resistance, hepatosteatosis, and cardiovascular complications.;Vernochet Cecile, Damilano Federico, Mourier Arnaud, Bezy Olivier, Mori Marcelo A, Smyth Graham, Rosenzweig Anthony, Larsson Nils-Göran, Kahn C Ronald, ;2014;FASEB journal : official publication of the Federation of American Societies for Experimental Biology;28;4408-19; 25005176
Effects of the selective adenosine A2A receptor antagonist, SCH 412348, on the parkinsonian phenotype of MitoPark mice.;Smith Karen M, Browne Susan E, Jayaraman Srinivasan, Bleickardt Carina J, Hodge Lisa M, Lis Edward, Yao Leon, Rittle Sunday L, Innocent Nathalie, Mullins Deborra E, Boykow George, Reynolds Ian J, Hill David, Parker Eric M, Hodgson Robert A, ;2014;European journal of pharmacology;728;31-8; 24486705
Loss of dopamine disrupts circadian rhythms in a mouse model of Parkinson's disease.;Fifel Karim, Cooper Howard M, ;2014;Neurobiology of disease;71;359-69; 25171792
Molecular mechanisms underlying protective effects of quercetin against mitochondrial dysfunction and progressive dopaminergic neurodegeneration in cell culture and MitoPark transgenic mouse models of Parkinson's Disease.;Ay Muhammet, Luo Jie, Langley Monica, Jin Huajun, Anantharam Vellareddy, Kanthasamy Arthi, Kanthasamy Anumantha G, ;2017;Journal of neurochemistry;141;766-782; 28376279
A switch in metabolism precedes increased mitochondrial biogenesis in respiratory chain-deficient mouse hearts.;Hansson Anna, Hance Nicole, Dufour Eric, Rantanen Anja, Hultenby Kjell, Clayton David A, Wibom Rolf, Larsson Nils-Göran, ;2004;Proceedings of the National Academy of Sciences of the United States of America;101;3136-41; 14978272
Cognitive dysfunction precedes the onset of motor symptoms in the MitoPark mouse model of Parkinson's disease.;Li Xiuhua, Redus Laney, Chen Cang, Martinez Paul A, Strong Randy, Li Senlin, O'Connor Jason C, ;2013;PloS one;8;e71341; 23977020
Release parameters during progressive degeneration of dopamine neurons in a mouse model reveal earlier impairment of spontaneous than forced behaviors.;Chen Yuan-Hao, Hsieh Tsung-Hsun, Kuo Tung-Tai, Kao Jen-Hsin, Ma Kuo-Hsing, Huang Eagle Yi-Kung, Chou Yu-Ching, Olson Lars, Hoffer Barry J, ;2019;Journal of neurochemistry;150;56-73; 30933310
Mito-Apocynin Prevents Mitochondrial Dysfunction, Microglial Activation, Oxidative Damage, and Progressive Neurodegeneration in MitoPark Transgenic Mice.;Langley Monica, Ghosh Anamitra, Charli Adhithiya, Sarkar Souvarish, Ay Muhammet, Luo Jie, Zielonka Jacek, Brenza Timothy, Bennett Brian, Jin Huajun, Ghaisas Shivani, Schlichtmann Benjamin, Kim Dongsuk, Anantharam Vellareddy, Kanthasamy Arthi, Narasimhan Balaji, Kalyanaraman Balaraman, Kanthasamy Anumantha G, ;2017;Antioxidants & redox signaling;27;1048-1066; 28375739
Altered glutamate release in the dorsal striatum of the MitoPark mouse model of Parkinson's disease.;Farrand Ariana Q, Gregory Rebecca A, Bäckman Cristina M, Helke Kristi L, Boger Heather A, ;2016;Brain research;1651;88-94; 27659966
Dopaminergic Neurons Exhibit an Age-Dependent Decline in Electrophysiological Parameters in the MitoPark Mouse Model of Parkinson's Disease.;Branch Sarah Y, Chen Cang, Sharma Ramaswamy, Lechleiter James D, Li Senlin, Beckstead Michael J, ;2016;The Journal of neuroscience : the official journal of the Society for Neuroscience;36;4026-37; 27053209
PTEN deletion enhances survival, neurite outgrowth and function of dopamine neuron grafts to MitoPark mice.;Zhang YaJun, Granholm Ann-Charlotte, Huh Kyounghee, Shan Lufei, Diaz-Ruiz Oscar, Malik Nasir, Olson Lars, Hoffer Barry J, Lupica Carl R, Hoffman Alexander F, Bäckman Cristina M, ;2012;Brain : a journal of neurology;135;2736-49; 22961549
Delayed Dopamine Dysfunction and Motor Deficits in Female Parkinson Model Mice.;Chen Yuan-Hao, Wang Vicki, Huang Eagle Yi-Kung, Chou Yu-Ching, Kuo Tung-Tai, Olson Lars, Hoffer Barry J, ;2019;International journal of molecular sciences;20;261-9; 31835787
L-Dopa induced dyskinesias in Parkinsonian mice: Disease severity or L-Dopa history.;Shan Lufei, Diaz Oscar, Zhang Yajun, Ladenheim Bruce, Cadet Jean-Lud, Chiang Yung-Hsiao, Olson Lars, Hoffer Barry J, Bäckman Cristina M, ;2015;Brain research;1618;106-10; 26086365
Manipulation of mitochondrial DNA gene expression in the mouse.;Silva José P, Larsson Nils-Göran, ;2002;Biochimica et biophysica acta;1555;146301; 12206900
Voluntary exercise delays progressive deterioration of markers of metabolism and behavior in a mouse model of Parkinson's disease.;Lai Jing-Huei, Chen Kai-Yun, Wu John Chung-Che, Olson Lars, Brené Stefan, Huang Chi-Zong, Chen Yen-Hua, Kang Shuo-Jhen, Ma Kuo-Hsing, Hoffer Barry J, Hsieh Tsung-Hsun, Chiang Yung-Hsiao, ;2019;Brain research;1720;429-436.e4; 31226324
Increased Total mtDNA Copy Number Cures Male Infertility Despite Unaltered mtDNA Mutation Load.;Jiang Min, Kauppila Timo Eino Sakari, Motori Elisa, Li Xinping, Atanassov Ilian, Folz-Donahue Kat, Bonekamp Nina Anna, Albarran-Gutierrez Sara, Stewart James Bruce, Larsson Nils-Göran, ;2017;Cell metabolism;26;15066-71; 28768180
Increased mitochondrial mass in mitochondrial myopathy mice.;Wredenberg Anna, Wibom Rolf, Wilhelmsson Hans, Graff Caroline, Wiener Heidi H, Burden Steven J, Oldfors Anders, Westerblad Håkan, Larsson Nils-Göran, ;2002;Proceedings of the National Academy of Sciences of the United States of America;99;231-6; 12417746
Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice.;Larsson N G, Wang J, Wilhelmsson H, Oldfors A, Rustin P, Lewandoski M, Barsh G S, Clayton D A, ;1998;Nature genetics;18;12937-42; 9500544
Impaired mitochondrial transport and Parkin-independent degeneration of respiratory chain-deficient dopamine neurons in vivo.;Sterky Fredrik H, Lee Seungmin, Wibom Rolf, Olson Lars, Larsson Nils-Göran, ;2011;Proceedings of the National Academy of Sciences of the United States of America;108;e0151884; 21768369
Multimodal MRI Evaluation of the MitoPark Mouse Model of Parkinson's Disease.;Cong Linlin, Muir Eric R, Chen Cang, Qian Yusheng, Liu Jingwei, Biju K C, Clark Robert A, Li Senlin, Duong Timothy Q, ;2016;PloS one;11;; 27003179

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Requesting frozen sperm or embryos is generally advisable wherever possible, in order to minimise the shipment of live mice.

Frozen sperm. Delivered in 4 weeks (after paperwork in place). €1740^*
Rederivation of mice from frozen stock, delivery time available upon request . €3880^*

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B6N.129-Tfamtm1Lrsn/Kctt