Francisca Diaz

Summary

Affiliation: University of Miami
Country: USA

Publications

  1. doi request reprint Cytochrome c oxidase deficiency: patients and animal models
    Francisca Diaz
    Department of Neurology, University of Miami Miller School of Medicine, 1095 NW 14th Terrace, Miami, Florida 33136, USA
    Biochim Biophys Acta 1802:100-10. 2010
  2. pmc A defect in the mitochondrial complex III, but not complex IV, triggers early ROS-dependent damage in defined brain regions
    Francisca Diaz
    Department of Neurology, University of Miami, Miller School of Medicine, Miami, FL 33136, USA
    Hum Mol Genet 21:5066-77. 2012
  3. pmc Cells lacking Rieske iron-sulfur protein have a reactive oxygen species-associated decrease in respiratory complexes I and IV
    Francisca Diaz
    Department of Neurology, University of Miami, Miller School of Medicine, Miami, Florida, USA
    Mol Cell Biol 32:415-29. 2012
  4. pmc Mitochondrial disorders caused by mutations in respiratory chain assembly factors
    Francisca Diaz
    Department of Neurology, University of Miami Miller School of Medicine, Miami, Florida, USA
    Semin Fetal Neonatal Med 16:197-204. 2011
  5. doi request reprint Endurance exercise is protective for mice with mitochondrial myopathy
    Tina Wenz
    Dept of Neurology, Miller School of Medicine, Univ of Miami, 1095 NW 14th Terrace, Miami, FL 33136, USA
    J Appl Physiol (1985) 106:1712-9. 2009
  6. pmc Cytochrome c oxidase is required for the assembly/stability of respiratory complex I in mouse fibroblasts
    Francisca Diaz
    Department of Neurology, University of Miami School of Medicine, 1095 NW 14 Terrace, Miami, FL 33136, USA
    Mol Cell Biol 26:4872-81. 2006
  7. pmc Cytochrome c oxidase deficiency in neurons decreases both oxidative stress and amyloid formation in a mouse model of Alzheimer's disease
    Hirokazu Fukui
    Neuroscience Program and Department of Neurology, University of Miami Miller School of Medicine, Miami, FL 33136, USA
    Proc Natl Acad Sci U S A 104:14163-8. 2007
  8. pmc Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype
    Tina Wenz
    Department of Neurology, University of Miami, School of Medicine, Miami, FL 33136, USA
    Cell Metab 8:249-56. 2008
  9. pmc Mouse models of oxidative phosphorylation defects: powerful tools to study the pathobiology of mitochondrial diseases
    Alessandra Torraco
    Department of Neurology, University of Miami Miller School of Medicine, 1095 NW 14th Terrace, Miami, FL 33136, USA
    Biochim Biophys Acta 1793:171-80. 2009
  10. pmc PGC-1alpha/beta induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders
    Sarika Srivastava
    Department of Neurology, University of Miami School of Medicine, Miami, FL 33136, USA
    Hum Mol Genet 18:1805-12. 2009

Collaborators

Detail Information

Publications21

  1. doi request reprint Cytochrome c oxidase deficiency: patients and animal models
    Francisca Diaz
    Department of Neurology, University of Miami Miller School of Medicine, 1095 NW 14th Terrace, Miami, Florida 33136, USA
    Biochim Biophys Acta 1802:100-10. 2010
    ..These animal models, ranging from invertebrates to mammals, in most cases mimic the pathological features of the human diseases...
  2. pmc A defect in the mitochondrial complex III, but not complex IV, triggers early ROS-dependent damage in defined brain regions
    Francisca Diaz
    Department of Neurology, University of Miami, Miller School of Medicine, Miami, FL 33136, USA
    Hum Mol Genet 21:5066-77. 2012
    ..These findings illustrate how specific respiratory chain defects have distinct molecular mechanisms, leading to distinct pathologies, akin to the clinical heterogeneity observed in patients with mitochondrial diseases...
  3. pmc Cells lacking Rieske iron-sulfur protein have a reactive oxygen species-associated decrease in respiratory complexes I and IV
    Francisca Diaz
    Department of Neurology, University of Miami, Miller School of Medicine, Miami, Florida, USA
    Mol Cell Biol 32:415-29. 2012
    ..Our data suggest that the stability of CI, CIV, and supercomplexes is regulated by ROS in the context of defective oxidative phosphorylation...
  4. pmc Mitochondrial disorders caused by mutations in respiratory chain assembly factors
    Francisca Diaz
    Department of Neurology, University of Miami Miller School of Medicine, Miami, Florida, USA
    Semin Fetal Neonatal Med 16:197-204. 2011
    ..Here, we describe assembly factors or chaperones that are required for respiratory chain complex assembly and their clinical relevance...
  5. doi request reprint Endurance exercise is protective for mice with mitochondrial myopathy
    Tina Wenz
    Dept of Neurology, Miller School of Medicine, Univ of Miami, 1095 NW 14th Terrace, Miami, FL 33136, USA
    J Appl Physiol (1985) 106:1712-9. 2009
    ..Thus stimulating residual oxidative phosphorylation function in the affected muscle by inducing mitochondrial biogenesis through endurance exercise might offer a valuable therapeutic intervention for mitochondrial myopathy patients...
  6. pmc Cytochrome c oxidase is required for the assembly/stability of respiratory complex I in mouse fibroblasts
    Francisca Diaz
    Department of Neurology, University of Miami School of Medicine, 1095 NW 14 Terrace, Miami, FL 33136, USA
    Mol Cell Biol 26:4872-81. 2006
    ..These results suggest that in rapidly dividing cells, complex IV is required for complex I assembly or stability...
  7. pmc Cytochrome c oxidase deficiency in neurons decreases both oxidative stress and amyloid formation in a mouse model of Alzheimer's disease
    Hirokazu Fukui
    Neuroscience Program and Department of Neurology, University of Miami Miller School of Medicine, Miami, FL 33136, USA
    Proc Natl Acad Sci U S A 104:14163-8. 2007
    ..Collectively, our results suggest that, contrary to previous models, a defect in neuronal COX does not increase oxidative damage nor predispose for the formation of amyloidgenic amyloid precursor protein fragments...
  8. pmc Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype
    Tina Wenz
    Department of Neurology, University of Miami, School of Medicine, Miami, FL 33136, USA
    Cell Metab 8:249-56. 2008
    ..Thus, induction of mitochondrial biogenesis through pharmacological or metabolic modulation of the PPAR/PGC-1alpha pathway promises to be an effective therapeutic approach for mitochondrial disorders...
  9. pmc Mouse models of oxidative phosphorylation defects: powerful tools to study the pathobiology of mitochondrial diseases
    Alessandra Torraco
    Department of Neurology, University of Miami Miller School of Medicine, 1095 NW 14th Terrace, Miami, FL 33136, USA
    Biochim Biophys Acta 1793:171-80. 2009
    ..In this review we discuss the main mouse models of mitochondrial disease developed, focusing on the ones that directly affect the OXPHOS system...
  10. pmc PGC-1alpha/beta induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders
    Sarika Srivastava
    Department of Neurology, University of Miami School of Medicine, Miami, FL 33136, USA
    Hum Mol Genet 18:1805-12. 2009
    ..These results reinforce the concept that increased mitochondrial biogenesis is a promising venue for the treatment of mitochondrial diseases...
  11. pmc Role of cytochrome C in apoptosis: increased sensitivity to tumor necrosis factor alpha is associated with respiratory defects but not with lack of cytochrome C release
    Uma D Vempati
    University of Miami Miller School of Medicine, 1095 NW 14th Terrace, Miami, FL 33136, USA
    Mol Cell Biol 27:1771-83. 2007
    ..These studies underscore the importance of oxidative phosphorylation and apoptosome function to both the intrinsic and extrinsic apoptotic pathways...
  12. pmc Mice lacking COX10 in skeletal muscle recapitulate the phenotype of progressive mitochondrial myopathies associated with cytochrome c oxidase deficiency
    Francisca Diaz
    Department of Neurology, The Miami Project to Cure Paralysis, University of Miami Miller School of Medicine, 1095 NW 14th Terrace, Miami, FL 33136, USA
    Hum Mol Genet 14:2737-48. 2005
    ..This COX10 KO mouse allowed us to correlate the muscle function with residual COX activity, an estimate that can help predict the progression pattern of human mitochondrial myopathies...
  13. pmc Mitochondrial biogenesis and turnover
    Francisca Diaz
    Department of Neurology, University of Miami, Miller School of Medicine, 1095 NW 14th Terrace, Miami, FL 33136, USA
    Cell Calcium 44:24-35. 2008
    ..Mitochondrial malfunction or defects in any of the many pathways involved in mitochondrial biogenesis can lead to degenerative diseases and possibly play an important part in aging...
  14. pmc Techniques and pitfalls in the detection of pathogenic mitochondrial DNA mutations
    Carlos T Moraes
    Department of Neurology, University of Miami School of Medicine, Miami, Florida 33136, USA
    J Mol Diagn 5:197-208. 2003
    ..To address these problems, novel approaches were developed and are discussed in this review...
  15. pmc Evaluation of the mitochondrial respiratory chain and oxidative phosphorylation system using blue native gel electrophoresis
    Francisca Diaz
    University of Miami Miller School of Medicine, Miami, Florida, USA
    Curr Protoc Hum Genet . 2009
    ....
  16. pmc Evaluation of the mitochondrial respiratory chain and oxidative phosphorylation system using polarography and spectrophotometric enzyme assays
    Antoni Barrientos
    University of Miami Miller School of Medicine, Miami, Florida, USA
    Curr Protoc Hum Genet . 2009
    ..Based on the methods described elsewhere, the assays describe methods that form a biochemical characterization of the OXPHOS system in cells and mitochondria isolated from cultured cells or tissues...
  17. pmc Evaluation of the mitochondrial respiratory chain and oxidative phosphorylation system using yeast models of OXPHOS deficiencies
    Flavia Fontanesi
    University of Miami Miller School of Medicine, Miami, Florida, USA
    Curr Protoc Hum Genet . 2009
    ..Several approaches for the assessment of the OXPHOS system enzymes have been developed. Based on the methods described elsewhere, this unit describes the creation and study of yeast models of mitochondrial OXPHOS deficiencies...
  18. ncbi request reprint Mitochondrial DNA structure and function
    Carlos T Moraes
    Department of Neurology, University of Miami School of Medicine, Miami, Florida 33136, USA
    Int Rev Neurobiol 53:3-23. 2002
  19. doi request reprint Synergistic inhibitory effect of nicotine plus oral contraceptive on mitochondrial complex-IV is mediated by estrogen receptor-β in female rats
    Ami P Raval
    Cerebral Vascular Disease Research Laboratories, Leonard M Miller School of Medicine, University of Miami, Miami, FL 33136, USA
    J Neurochem 121:157-67. 2012
    ..Overall, these results suggest that mitochondrial ER-β loss is responsible for mitochondrial malfunction after NOC...
  20. doi request reprint Oral contraceptives and nicotine synergistically exacerbate cerebral ischemic injury in the female brain
    Ami P Raval
    Cerebral Vascular Disease Research Center, Department of Neurology, Leonard M Miller School of Medicine, University of Miami, Two Story Lab TSL, Room 230A, 1420 NW 9th Avenue, Miami, FL, 33101, USA
    Transl Stroke Res 4:402-12. 2013
    ..In the present review we highlight possible mechanisms by which OC and nicotine inhibits estrogen signaling that could be responsible for severe ischemic damage in females. ..
  21. pmc Human mitochondrial DNA with large deletions repopulates organelles faster than full-length genomes under relaxed copy number control
    Francisca Diaz
    Department of Neurology, University of Miami, School of Medicine, Miami, FL 33136, USA
    Nucleic Acids Res 30:4626-33. 2002
    ..Taken together, our results indicate that, under relaxed copy number control, DeltamtDNAs repopulate mitochondria more efficiently than full-length genomes...