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Cytochrome c oxidase (COX) biogenesis in health and disease
COX is the terminal oxidase of the respiratory chain. COX deficiency is the major cause of mitochondrial encephalomyopathies in humans. Our long-term goal is to attain a complete understanding of the pathways leading to COX assembly and their components as a prerequisite to the development of therapies for the management of disorders associated with COX deficiencies.
Mitochondrial physiology and biogenesis in cellular stress and aging
Metabolic and mitochondrial abnormalities are a prominent feature of aging and neurodegeneration. However, the literature reports conflicting results concerning the extent and causality of the aging associated aerobic energy production decline and mitochondrial ROS-induced damage, as well as their interplay with nutritional cues. Single cell models have provided key information concerning mechanisms of aging and neurodegeneration.
Mitochondrial translation and biogenesis of the mitochondrial translational apparatus in yeast and mammalian cells.
Over the last few years we have become very interested in the biogenesis of the mitochondrial ribosomes. The process is complicated by the fact that the two mitoribosomal RNAs (rRNAs) are universally mitochondrion-encoded whereas all ribosomal proteins (with a single exception in yeast) are encoded in the nuclear DNA. Its biomedical importance is highlighted by the fact that mutations affecting genes encoding mitochondrial ribosomal subunits are responsible for infantile multisystemic mitochondrial diseases, frequently involving encephalomyopathy and hypertrophic cardiomyopathy.
The biosynthetic pathway of human mitochondrial respirasomes
The mitochondrial respiratory chain (MRC) assembly and function involve the organization of its constitutive complexes in supercomplexes or respirasomes. It is believed that supercomplexes have an important functional role in cellular bioenergetics by optimizing electron transfer, proton pumping and controlling the formation of reactive intermediates. We are investigating the players and mechanisms involved in MRC supercomplex assembly using yeast and cultured human cells as research models.
Andrea Lynema presents a poster at the Neuroscience Research Day
Hosted by UM’s Neuroscience Graduate Program, the 23rd Annual Neuroscience Research Day took place on Friday, November 7 to showcase and promote neuroscience in South Florida and provide a venue for current and future neuroscientists to share ideas and develop networking opportunities. Andrea is a student from the Neuroscience graduate program and presented a poster […]
Manuscript Publication in The FASEB Journal by Dr Jingjing Liu.
A mitochondrial CO2-adenylyl cyclase-cAMP signalosome controls yeast normoxic cytochrome c oxidase activity” by Ken C. Hess, Jingjing Liu J, Manfredi G, Mühlschlegel FA, Buck J, Levin LR and Antoni Barrientos. Dr Jingjing Liu obtained her PhD in the lab in 2013 and is currently following postdoctoral training at Duke University. Mitochondria respond to changes in […]
Manuscript Publication in Human Molecular Genetics by Dr. Myriam Bourens
“Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase” by Myriam Bourens, Boulet A, Leary SC, and Antoni Barrientos. We have used small interference RNA and transcription activator-like effector nucleases (TALENs) technology to create knockdown and knockout human cell lines, respectively, to study the function of […]
Dr. Iliana Soto, Dr. Myriam Bourens and Dr. Antoni Barrientos presented posters at the Euromit meeting in Tempere (Finland)
The posters presented highlighted our research on the assembly of mitochondrial respiratory chain enzymes and biogenesis of the mitochondrial ribosomes in yeast and human cells.
Dr. Flavia Fontanesi receives The University of Miami Miller School of Medicine Stanley J. Glaser Research Award, which honors Miller School’s Rising Stars
The Stanley J. Glaser Foundation Research Awards are intended to provide seed dollars for young investigators and position them to compete successfully for major federal grants.
Dr. Flavia Fontanesi receives a Research Grant from the American Heart Association (AHA)
Dr. Fontanesi has received a 4-year grant to study the assembly of mitochondrial respiratory chain enzymes in the yeast Saccharomyces cerevisiae.
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