# positions[].organizations[].parent[].label
Texas A&M Engineering Experiment Station (TEES)

# positions[].organizations[].parent[].label
College of Agriculture and Life Sciences

# overview
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.

# keywords[]
1.1 Normal Biological Development And Functioning

# keywords[]
1.1 Normal Biological Development And Functioning

# keywords[]
Electron Transport Chain Complex Proteins

# keywords[]
Electron Transport Chain Complex Proteins

# keywords[]
1.1 Normal Biological Development And Functioning

# educationAndTraining[].organization.label
Wayne State University - (Detroit, Michigan, United States)

# educationAndTraining[].organization.label
Maharaja Sayajirao University of Baroda - (Vadodara, Gujarat, India)

# educationAndTraining[].organization.label
Mohanlal Sukhadia University - (Udaipur, India)

# publications[].label
FDX1 is required for the biogenesis of mitochondrial cytochrome c oxidase in mammalian cells.

# publications[].label
Absence of cardiolipin results in temperature sensitivity, respiratory defects, and mitochondrial DNA instability independent of pet56.

# publications[].label
Loss of cardiolipin leads to perturbation of mitochondrial and cellular iron homeostasis.

# publications[].label
Binding of 10-N-nonyl acridine orange to cardiolipin-deficient yeast cells: implications for assay of cardiolipin.

# publications[].label
Elesclomol elevates cellular and mitochondrial iron levels by delivering copper to the iron import machinery.

# publications[].label
Correction: Mitochondrial and nuclear genomic responses to loss of LRPPRC expression.

# publications[].label
Meclizine Preconditioning Protects the Kidney Against Ischemia-Reperfusion Injury.

# publications[].label
Mitochondrial copper in human genetic disorders.

# publications[].label
COA6 Is Structurally Tuned to Function as a Thiol-Disulfide Oxidoreductase in Copper Delivery to Mitochondrial Cytochrome c Oxidase.

# publications[].label
Separation of yeast phospholipids using one-dimensional thin-layer chromatography.

# publications[].label
MICU1 encodes a mitochondrial EF hand protein required for Ca(2+) uptake.

# publications[].label
Molecular nature and physiological role of the mitochondrial calcium uniporter channel.

# publications[].label
Mitochondrial disease genes COA6, COX6B and SCO2 have overlapping roles in COX2 biogenesis.

# publications[].label
A genome wide copper-sensitized screen identifies novel regulators of mitochondrial cytochrome c oxidase activity

# publications[].label
FDX1-dependent and independent mechanisms of elesclomol-mediated intracellular copper delivery.

# publications[].publisher
Proceedings of the National Academy of Sciences

# publications[].label
Evolution of reduced pre-adult viability and larval growth rate in laboratory populations of Drosophila melanogaster selected for shorter development time.

# publications[].label
The Role of COA6 in the Mitochondrial Copper Delivery Pathway to Cytochrome c Oxidase.

# publications[].label
Copper supplementation restores cytochrome c oxidase assembly defect in a mitochondrial disease model of COA6 deficiency.

# publications[].label
A computational screen for regulators of oxidative phosphorylation implicates SLIRP in mitochondrial RNA homeostasis.

# publications[].label
Mitochondrial and nuclear genomic responses to loss of LRPPRC expression.

# publications[].label
COA6 is Structurally Tuned to Function as a Thiol-Disulfide Oxidoreductase in Copper Delivery to Mitochondrial Cytochrome c Oxidase

# publications[].label
MCUR1 Is a Scaffold Factor for the MCU Complex Function and Promotes Mitochondrial Bioenergetics.

# publications[].label
Synthetic lethal interaction of the mitochondrial phosphatidylethanolamine and cardiolipin biosynthetic pathways in Saccharomyces cerevisiae.

# publications[].label
Choline restores respiration in Psd1-deficient yeast by replenishing mitochondrial phosphatidylethanolamine.

# publications[].label
Cellular functions of cardiolipin in yeast.

# publications[].label
Editorial: Function and formation of mitochondrial metalloproteome.

# publications[].label
The role of nonbilayer phospholipids in mitochondrial structure and function.

# publications[].label
A yeast suppressor screen links Coa4 to the mitochondrial copper delivery pathway for cytochrome c oxidase.

# publications[].label
Yeast homologs of human MCUR1 regulate mitochondrial proline metabolism.

# publications[].label
Mitochondrial dysfunction reactivates -fetoprotein expression that drives copper-dependent immunosuppression in mitochondrial disease models.

# publications[].publisher
American Society for Clinical Investigation

# publications[].label
Mitochondrial phospholipid metabolism in health and disease.

# publications[].label
Nutrient-sensitized screening for drugs that shift energy metabolism from mitochondrial respiration to glycolysis.

# publications[].label
Mitochondrial dysfunctions in barth syndrome.

# publications[].label
An essential role for cardiolipin in the stability and function of the mitochondrial calcium uniporter.

# publications[].publisher
Proceedings of the National Academy of Sciences

# publications[].label
Specific requirements of nonbilayer phospholipids in mitochondrial respiratory chain function and formation.

# publications[].publisher
American Society for Cell Biology (ASCB)

# publications[].label
Repurposing elesclomol, an investigational drug for the treatment of copper metabolism disorders.

# publications[].label
Cardiolipin biosynthesis and mitochondrial respiratory chain function are interdependent.

# publications[].label
Noncanonical role of singleminded-2s in mitochondrial respiratory chain formation in breast cancer.

# publications[].label
Meclizine is neuroprotective in models of Huntington's disease.

# publications[].label
Meclizine inhibits mitochondrial respiration through direct targeting of cytosolic phosphoethanolamine metabolism.

# publications[].label
Elesclomol alleviates Menkes pathology and mortality by escorting Cu to cuproenzymes in mice.

# publications[].publisher
American Association for the Advancement of Science (AAAS)

# publications[].label
Cardiolipin deficiency leads to the destabilization of mitochondrial magnesium channel MRS2 in Barth syndrome.

# publications[].label
Elesclomol restores mitochondrial function in genetic models of copper deficiency.

# publications[].publisher
Proceedings of the National Academy of Sciences

# publications[].label
MCU-complex-mediated mitochondrial calcium signaling is impaired in Barth syndrome.

# publications[].label
Mssbauer study and modeling of iron import and trafficking in human jurkat cells.

# publications[].label
A genome-wide copper-sensitized screen identifies novel regulators of mitochondrial cytochrome c oxidase activity.

# publications[].label
Mitochondrial membrane biogenesis: phospholipids and proteins go hand in hand.

# publications[].label
Correction: A Computational Screen for Regulators of Oxidative Phosphorylation Implicates SLIRP in Mitochondrial RNA Homeostasis

# publications[].label
Mitochondrial dysfunction triggers secretion of the immunosuppressive factor -fetoprotein

# publications[].label
Connecting copper and cancer: from transition metal signalling to metalloplasia.

# researcherOn[].label
Discovery and Characterization of Novel Regulators of Mitochondrial Copper Homeostasis

# researcherOn[].awardedBy.label
National Institutes of Health - (Bethesda, Maryland, United States)

# researcherOn[].awardedBy.preferredLabel
DHHS-NIH-National Institute of General Medical Science

# researcherOn[].label
Molecular Mechanisms of Copper Delivery to Mitochondrial Cytochrome c Oxidase

# researcherOn[].awardedBy.label
National Institutes of Health - (Bethesda, Maryland, United States)

# researcherOn[].awardedBy.preferredLabel
DHHS-NIH-National Institute of General Medical Science

# researcherOn[].label
Correcting mitochondrial defects in Barth syndrome models by targeting phosphatidylethanolamine metabolism

# researcherOn[].awardedBy.label
American Heart Association - (New York, New York, United States)

# researcherOn[].label
Epigenetics of the Aging Astrocyte: Implications for Stroke

# researcherOn[].awardedBy.label
National Institutes of Health - (Bethesda, Maryland, United States)

# streetAddress
Texas A&M University; Biochemistry & Biophysics; 3474 TAMU

# schools[]
Texas A&M Engineering Experiment Station (TEES)

# schools[]
College of Agriculture and Life Sciences

# inTheNews[].label
Promising study offers hope for Menkes disease treatment

# inTheNews[].label
New link between calcium and cardiolipin in heart defects

# etdChairOf[].label
Baker, Charli D (2017-05). Phospholipid Requirements for Mitochondrial Respiratory Chain Function. Doctoral Dissertation.

# etdChairOf[].label
Pratt, Anthony Thomas (2015-08). Functional and Regulatory Studies of Cytochrome c Oxidase Assembly Factor 6. Master's Thesis.