Despite the fundamental role of the mitochondrion in cellular energy production and its involvement in numerous human diseases, we still do not know the function of nearly 20% of the known mitochondrial proteins. My laboratory applies genomic, genetic, and biochemical tools to uncover the role of these uncharacterized proteins in the mitochondrial respiratory chain (MRC) biogenesis. MRC is the main site of cellular respiration and energy production and since the core components of the MRC are evolutionarily conserved, we reason that the assembly factors required to build the MRC should also be conserved. Therefore, we utilize multiple models systems, including yeast, zebrafish, and human cell lines, to determine the role of these conserved, uncharacterized mitochondrial proteins in bioenergetics, organismal development, and human disease pathogenesis.
Another poorly understood aspect of the mitochondrial energy metabolism is the role of phospholipids in maintaining the structural and functional integrity of the MRC. Although it is well known that the MRC is localized in the inner mitochondrial membrane, how the unique lipid milieu of the mitochondrial membrane influences the assembly and activity of the MRC is not fully understood. We have constructed yeast mutants with defined mitochondrial phospholipid compositions to systematically determine each lipid's role in MRC assembly and activity. Ultimately, defining the roles of mitochondrial proteins and phospholipids will allow us to develop better diagnostic and therapeutic options for human disorders resulting from mitochondrial dysfunction.
- Ph.D. in , Wayne State University - (Detroit, Michigan, United States) 2005
- M.Sc. in Biochemistry, Maharaja Sayajirao University of Baroda - (Vadodara, Gujarat, India) 1997
- B.Sc. in , Mohanlal Sukhadia University - (Udaipur, India) 1995
- Iadarola, D. M., Ball, W. B., Trivedi, P. P., Fu, G., Nan, B., & Gohil, V. M. (2020). yyy Vps39 is required for ethanolamine-stimulated elevation in mitochondrial phosphatidylethanolamine. Biochimica et Biophysica Acta - Molecular and Cell Biology of Lipids. 1865(6), 158655-158655.
- Guthrie, L. M., Soma, S., Yuan, S., Silva, A., Zulkifli, M., Snavely, T. C., ... Sacchettini, J. C. (2020). Elesclomol alleviates Menkes pathology and mortality by escorting Cu to cuproenzymes in mice.. Science (New York, N.Y.). 368(6491), 620-625.
- Soma, S., Morgada, M. N., Naik, M. T., Boulet, A., Roesler, A. A., Dziuba, N., ... Gohil, V. M. (2019). COA6 Is Structurally Tuned to Function as a Thiol-Disulfide Oxidoreductase in Copper Delivery to Mitochondrial Cytochrome c Oxidase. CELL REPORTS. 29(12), 4114-+.
- Ghosh, S., Iadarola, D. M., Ball, W. B., & Gohil, V. M. (2019). Mitochondrial dysfunctions in barth syndrome. IUBMB Life. 71(7), 791-801.
- Soma, S., Latimer, A. J., Chun, H., Vicary, A. C., Timbalia, S. A., Boulet, A., ... Gohil, V. M. (2018). Elesclomol restores mitochondrial function in genetic models of copper deficiency. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES. 115(32), 8161-8166.
- Baker, Charli D (2017-05). Phospholipid Requirements for Mitochondrial Respiratory Chain Function. (Doctoral Dissertation)
- Pratt, Anthony Thomas (2015-08). Functional and Regulatory Studies of Cytochrome c Oxidase Assembly Factor 6. (Master's Thesis)