Modelling Mitochondrial Dysfunction in Mice

被引:13
作者
Dogan, S. A. [1 ]
Trifunovic, A. [1 ,2 ]
机构
[1] Univ Cologne, Cologne, Germany
[2] Karolinska Inst, Div Metab Dis, Dept Lab Med, Stockholm, Sweden
关键词
Mitochondrial diseases; mtDNA; Mouse models; Encephalomyopathies; Aging; TRANSCRIPTION FACTOR; DNA; MUTATIONS; GENE; EXPRESSION; MAINTENANCE; PHENOTYPES; DEPLETION; DEFECTS; SURF-1;
D O I
10.33549/physiolres.932179
中图分类号
Q4 [生理学];
学科分类号
071003 ;
摘要
Understanding mitochondrial role in normal physiology and pathological conditions has proven to be of high importance as mitochondrial dysfunction is connected with a number of disorders as well as some of the most common diseases (e. g. diabetes or Parkinson's disease). Modeling mitochondrial dysfunction has been difficult mainly due to unique features of mitochondrial genetics. Here we discuss some of the most important mouse models generated so far and lessons learned from them.
引用
收藏
页码:S61 / S70
页数:10
相关论文
共 43 条
[1]   Thymidine kinase 2 (H126N) knockin mice show the essential role of balanced deoxynucleotide pools for mitochondrial DNA maintenance [J].
Akman, Hasan O. ;
Dorado, Beatriz ;
Lopez, Luis C. ;
Garcia-Cazorla, Angeles ;
Vila, Maya R. ;
Tanabe, Lauren M. ;
Dauer, William T. ;
Bonilla, Eduardo ;
Tanji, Kurenai ;
Hirano, Michio .
HUMAN MOLECULAR GENETICS, 2008, 17 (16) :2433-2440
[2]   SEQUENCE AND ORGANIZATION OF THE HUMAN MITOCHONDRIAL GENOME [J].
ANDERSON, S ;
BANKIER, AT ;
BARRELL, BG ;
DEBRUIJN, MHL ;
COULSON, AR ;
DROUIN, J ;
EPERON, IC ;
NIERLICH, DP ;
ROE, BA ;
SANGER, F ;
SCHREIER, PH ;
SMITH, AJH ;
STADEN, R ;
YOUNG, IG .
NATURE, 1981, 290 (5806) :457-465
[3]  
BOURGERON T, 1993, J BIOL CHEM, V268, P19369
[4]   Epidemiology and treatment of mitochondrial disorders [J].
Chinnery, PF ;
Turnbull, DM .
AMERICAN JOURNAL OF MEDICAL GENETICS, 2001, 106 (01) :94-101
[5]   MELAS MUTATION IN MTDNA BINDING-SITE FOR TRANSCRIPTION TERMINATION FACTOR CAUSES DEFECTS IN PROTEIN-SYNTHESIS AND IN RESPIRATION BUT NO CHANGE IN LEVELS OF UPSTREAM AND DOWNSTREAM MATURE TRANSCRIPTS [J].
CHOMYN, A ;
MARTINUZZI, A ;
YONEDA, M ;
DAGA, A ;
HURKO, O ;
JOHNS, D ;
LAI, ST ;
NONAKA, I ;
ANGELINI, C ;
ATTARDI, G .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1992, 89 (10) :4221-4225
[6]   Extension of Drosophila Life Span by RNAi of the Mitochondrial Respiratory Chain [J].
Copeland, Jeffrey M. ;
Cho, Jaehyoung ;
Lo, Thomas, Jr. ;
Hur, Jae H. ;
Bahadorani, Sepehr ;
Arabyan, Tagui ;
Rabie, Jason ;
Soh, Jennifer ;
Walker, David W. .
CURRENT BIOLOGY, 2009, 19 (19) :1591-1598
[7]   Increased longevity and refractoriness to Ca2+-dependent neurodegeneration in Surf1 knockout mice [J].
Dell'Agnello, Carlotta ;
Leo, Sara ;
Agostino, Alessandro ;
Szabadkai, Gyorgy ;
Tiveron, Cecilia ;
Zulian, Alessandra ;
Prelle, Alessandro ;
Roubertoux, Pierre ;
Rizzuto, Rosario ;
Zeviani, Massimo .
HUMAN MOLECULAR GENETICS, 2007, 16 (04) :431-444
[8]   Rates of behavior and aging specified by mitochondrial function during development [J].
Dillin, A ;
Hsu, AL ;
Arantes-Oliveira, NA ;
Lehrer-Graiwer, J ;
Hsin, H ;
Fraser, AG ;
Kamath, RS ;
Ahringer, J ;
Kenyon, C .
SCIENCE, 2002, 298 (5602) :2398-2401
[9]   MITOCHONDRIAL MYOPATHIES [J].
DIMAURO, S ;
BONILLA, E ;
ZEVIANI, M ;
NAKAGAWA, M ;
DEVIVO, DC .
ANNALS OF NEUROLOGY, 1985, 17 (06) :521-538
[10]   Random Point Mutations with Major Effects on Protein-Coding Genes Are the Driving Force behind Premature Aging in mtDNA Mutator Mice [J].
Edgar, Daniel ;
Shabalina, Irina ;
Camara, Yolanda ;
Wredenberg, Anna ;
Calvaruso, Maria Antonietta ;
Nijtmans, Leo ;
Nedergaard, Jan ;
Cannon, Barbara ;
Larsson, Nils-Goeran ;
Trifunovic, Aleksandra .
CELL METABOLISM, 2009, 10 (02) :131-138