Contact image
Center for Mitochondrial and Epigenomic Medicine (CMEM)
Children’s Hospital of Philadelphia Research Institute
Colket Translational Research Building
3501 Civic Center Boulevard, Room 6100
Philadelphia, PA 
Email: This email address is being protected from spambots. You need JavaScript enabled to view it.
Phone: (267) 425-3072

Research Statement

Dr. Potluri's research is focused on identifying nuclear genes, through complementation, that can cause or increase the severity of mitochondrial disease phenotype and developing gene therapy strategies to treat mitochondrial diseases in mouse models. Specifically, he is interested in mitochondrial Oxidative phosphorylation and mitochondrial protein synthesis deficiencies. He has been developing high throughput viral vectors like MMLV based retroviral viral cDNA expression vectors and Adeno Assocaited Vius  based expression vectors for his studies. He extensively uses various bio chemical tools like Clark electrodes and Blue Native-Gel based protein chemistry techniques to understand the biology of mitochondria in his studies.

Selected Publications

  • Potluri   P, et. al., (2009) A novel NDUFA1 mutation leads to a progressive  mitochondrial complex I-specific neurodegenerative disease.  Mol Genet Metab. 94:189.
  • Potluri P., et. al.,  (2004) The role of the ESSS protein in the assembly of a functional and stable mammalian mitochondrial complex I (NADH-ubiquinone oxidoreductase). Eur J. Biochem 271: 3265.
  • Yadava N, Potluri P*, Scheffler IE. (2007) Investigations of the potential effects of phosphorylation of the MWFE and ESSS subunits on complex I activity and assembly Int J Biochem Cell Biol  40: 447.
  • Yadava N., Potluri P., Smith EN., Scheffler IE (2002). Species-specific and Mutant MWFE Proteins: Their effect on the assembly of a functional mammalian mitochondrial complex I. J. Biol Chem 277: 21221.
  • Broderick KE, Potluri P, Zhuang S, Scheffler IE, Sharma VS, Pilz RB  and Boss GR (2006) C yanide detoxification by the cobalamin precursor cobinamide. Exp Biol Med  231: 641-649.

PubMed Search 

Additional information