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Pinkert 2013 Abstract MiP2013

From Bioblast
Pinkert C, Parameshwaran K, Augsburger B, Steliou K, Trounce IA, Irwin MH (2013) Murine modeling of human mitochondrial disease pathogenesis. Mitochondr Physiol Network 18.08.

Link:

Pinkert C, Parameshwaran K, Augsburger B, Steliou K, Trounce IA, Irwin MH (2013)

Event: MiPNet18.08_MiP2013

Carl Pinkert

The ability to manipulate and regulate the expression of mitochondrial genes will provide a basis for developing innovative gene therapy paradigms. Our efforts to date have focused on four broad research categories pertaining to mitochondrial disease [1-5]. We have developed or are currently characterizing transmitochondrial mouse models of human disease including models for Parkinsonโ€™s disease and Complex I assembly, deficiencies and Leigh disease, and therapeutic intervention for mitochondrial based diseases.

Mitochondrial genome and mitochondrial gene transfer were both pursued in the creation of transmitochondrial mice (mice harboring modified and species-specific mitochondrial genes/genetics) and represent model systems in development. Efforts to define deficiencies in respiration and subsequent motor coordination skills focus on key behavioral and genetic testing endpoints. Evaluation of mitochondrial mechanisms regulating synaptic plasticity and cognition with an emphasis on Complex I deficits is also underway. Current studies with in vivo and genetically-engineered models, collectively, are geared toward development of human disease models and therapeutic strategies in human mitochondrial disorders with a direct relationship to animal development.

โ€ข Keywords: inborn mt-disease

โ€ข O2k-Network Lab: AU Melbourne Trounce IA


Labels: MiParea: mtDNA;mt-genetics, Genetic knockout;overexpression  Pathology: Neurodegenerative 

Organism: Mouse 

Preparation: Intact organism  Enzyme: Complex I 



MiP2013 


Affiliations and author contributions

1 - Auburn University, Alabama, USA;

2 - PhenoMatriX and Boston University, Massachusetts, USA;

3 - University of Melbourne, Australia.

Email: cap@auburn.edu


References

  1. Pinkert CA, Irwin MH, Johnson LW, Moffatt RJ (1997) Mitochondria transfer into mouse ova by microinjection. Transgenic Res 6: 379-383.
  2. McKenzie M, Trounce IA, Cassar CA, Pinkert CA (2004) Production of homoplasmic xenomitochondrial mice. Proc Natl Acad Sci USA 101: 1685-1690.
  3. Dunn DA, Pinkert CA (2012) Nuclear expression of a mitochondrial DNA gene: Mitochondrial targeting of allotopically expressed mutant ATP6 in transgenic mice. J Biomed Biotechnol 2012: 541245.
  4. Parameshwaran K, Irwin MH, Steliou K, Pinkert CA (2012) Protection by an antioxidant of rotenone-induced neuromotor decline, reactive oxygen species generation and cellular stress in mouse brain. Pharmacol Biochem Behav 101: 487-492.
  5. Irwin MH, Parameshwaran K, Pinkert CA (2013) Mouse models of mitochondrial Complex I dysfunction. Intl J Biochem Cell Biol 45: 34-40.