- Undergraduate Education
- B.Sc. Mohan Lal Sukhadia University, India (1995)
- Graduate Education
- M.Sc. Maharaja Sayajirao University of Baroda, India (1997)
- Ph.D. Wayne State University, Detroit (2005)
- Postdoc. Massachusetts General Hospital, Boston (2005-2008)
- Instructor in Medicine, Harvard Medical School, Boston (2008-2011)
- Joined Texas A&M in 2012
Mitochondrial Respiratory Chain Biogenesis
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.
Guthrie, LM, Soma, S, Yuan, S, Silva, A, Zulkifli, M, Snavely, TC et al.. Elesclomol alleviates Menkes pathology and mortality by escorting Cu to cuproenzymes in mice. Science. 2020;368 (6491):620-625.
Gohil, VM, Nilsson, R, Belcher-Timme, CA, Luo, B, Root, DE, Mootha, VK et al.. Correction: Mitochondrial and nuclear genomic responses to loss of LRPPRC expression. J. Biol. Chem. 2020;295 (16):5533.
Iadarola, DM, Basu Ball, W, Trivedi, PP, Fu, G, Nan, B, Gohil, VM et al.. Vps39 is required for ethanolamine-stimulated elevation in mitochondrial phosphatidylethanolamine. Biochim Biophys Acta Mol Cell Biol Lipids. 2020;1865 (6):158655.
Soma, S, Morgada, MN, Naik, MT, Boulet, A, Roesler, AA, Dziuba, N et al.. COA6 Is Structurally Tuned to Function as a Thiol-Disulfide Oxidoreductase in Copper Delivery to Mitochondrial Cytochrome c Oxidase. Cell Rep. 2019;29 (12):4114-4126.e5.
Ghosh, S, Iadarola, DM, Ball, WB, Gohil, VM. Mitochondrial dysfunctions in barth syndrome. IUBMB Life. 2019;71 (7):791-801.
Soma, S, Latimer, AJ, Chun, H, Vicary, AC, Timbalia, SA, Boulet, A et al.. Elesclomol restores mitochondrial function in genetic models of copper deficiency. Proc. Natl. Acad. Sci. U.S.A. 2018;115 (32):8161-8166.
Basu Ball, W, Baker, CD, Neff, JK, Apfel, GL, Lagerborg, KA, Žun, G et al.. Ethanolamine ameliorates mitochondrial dysfunction in cardiolipin-deficient yeast cells. J. Biol. Chem. 2018;293 (28):10870-10883.
Basu Ball, W, Neff, JK, Gohil, VM. The role of nonbilayer phospholipids in mitochondrial structure and function. FEBS Lett. 2018;592 (8):1273-1290.
Baker, CD, Basu Ball, W, Pryce, EN, Gohil, VM. Specific requirements of nonbilayer phospholipids in mitochondrial respiratory chain function and formation. Mol. Biol. Cell. 2016;27 (14):2161-71.
Tomar, D, Dong, Z, Shanmughapriya, S, Koch, DA, Thomas, T, Hoffman, NE et al.. MCUR1 Is a Scaffold Factor for the MCU Complex Function and Promotes Mitochondrial Bioenergetics. Cell Rep. 2016;15 (8):1673-85.