Characterization of Saccharomyces cerevisiae strains and human fibroblast cultures defectives in the biosynthesis of coenzyme Q

• Grant number: 12/11524-8
• Support Opportunities: Scholarships in Brazil - Doctorate (Direct)
• Start date: September 01, 2012
• End date: January 08, 2018
• Field of knowledge: Biological Sciences - Biochemistry - Metabolism and Bioenergetics
• Principal Investigator: Mario Henrique de Barros
• Grantee: Janaina Areias Paulela
• Host Institution: Instituto de Ciências Biomédicas (ICB). Universidade de São Paulo (USP). São Paulo , SP, Brazil

Abstract

The respiratory chain depends on electron carriers such as Coenzyme Q. Coenzyme Q is responsible for transferring electrons from NADH and FADH2 dehydrogenases to complex III. Although reduction of CoQ levels is identified in many mitochondrial disorders, just recently primary deficient cases of CoQ have been described. Reactive oxygen species overproduction was detected in cell lines of patients with CoQ primary deficiencies and the level of ROS production correlated with the severity of the CoQ defect. Mitochondrial diseases have been treated by CoQ administration, which is considered harmless and has improved the clinical symptoms in many patients, probably by lowering the levels of ROS being produced in dysfunctional mitochondria. (AU)

Academic Publications

(References retrieved automatically from State of São Paulo Research Institutions)

PAULELA, Janaina Areias. Characterization of saccharomyces cerevisiae strains deficient in the biosynthesis of Coenzyme Q.. 2018. Doctoral Thesis - Universidade de São Paulo (USP). Instituto de Ciências Biomédicas (ICB/SDI) São Paulo.