Bianca Seminotti, MSc, PhD

  • Research Scientist

Administrative Assistant: Shannon Marie Joseph

Seminotti is a scientist with over 15 years of experience in biomedical research in the field of inborn errors of metabolism. Expertise in cellular metabolism, mitochondria and cell and molecular biology, using different in vivo and in vitro models.

Education & Training

  • BS, Biomedicine, Federal University of Rio Grande do Sul, Brazil, 2009
  • MSc, Federal University of Rio Grande do Sul, Brazil, 2011
  • PhD, Federal University of Rio Grande do Sul, 2014

Selected Publications

Seminotti, Bianca, Grings M, Tucci P, Leipnitz G, Saso L. Nuclear Factor Erythroid-2-Related Factor 2 Signaling in the Neuropathophysiology of Inherited Metabolic Disorders. Frontiers in Cellular Neuroscience. 2021 Nov 26;15:785057. 

Seminotti, Bianca, Roginski AC, Zanatta Â, Amaral AU, Fernandes T, Spannenberger KP, da Silva LHR, Ribeiro RT, Leipnitz G, Wajner M. S-adenosylmethionine induces mitochondrial dysfunction, permeability transition pore opening and redox imbalance in subcellular preparations of rat liver. Journal of Bioenergetics and Biomembranes. 2021 Aug 4. doi: 10.1007/s10863-021-09914-3.

Seminotti, Bianca, Amaral AU, Grings M, Ribeiro CAJ, Leipnitz G, Wajner M. Lipopolysaccharide-Elicited Systemic Inflammation Induces Selective Vulnerability of Cerebral Cortex and Striatum of Developing Glutaryl-CoA Dehydrogenase Deficient (Gcdh-/-) Mice to Oxidative Stress. Neurotoxicity Research. 2020 Dec;38(4):1024-1036. doi: 10.1007/s12640-020-00291-0

Seminotti, Bianca, da Silva JC, Ribeiro RT, Leipnitz G, Wajner M. Free Radical Scavengers Prevent Argininosuccinic Acid-Induced Oxidative Stress in the Brain of Developing Rats: a New Adjuvant Therapy for Argininosuccinate Lyase Deficiency? Molecular Neurobiology. 2020 Feb;57(2):1233-1244. doi: 10.1007/s12035-019-01825-0. 

Wajner M, Amaral AU, Leipnitz G, Seminotti, Bianca. Pathogenesis of brain damage in glutaric acidemia type I: Lessons from the genetic mice model. International Journal of Developmental Neuroscince 2019 Nov;78:215-221. doi: 10.1016/j.ijdevneu.2019.05.005. 

Seminotti, Bianca, Leipnitz G, Karunanidhi A, Kochersperger C, Roginskaya VY, Basu S, Wang Y, Wipf P, Van Houten B, Mohsen AW, Vockley J. Mitochondrial energetics is impaired in very long-chain acyl-CoA dehydrogenase deficiency and can be rescued by treatment with mitochondria-targeted electron scavengers. Human Molecular Genetics 28: 928-941, 2019 doi: 10.1093/hmg/ddy403. 

Seminotti, Bianca, Zanatta Â, Ribeiro RT, da Rosa MS, Wyse ATS, Leipnitz G, Wajner M. Disruption of Brain Redox Homeostasis, Microglia Activation and Neuronal Damage Induced by Intracerebroventricular Administration of S-Adenosylmethionine to Developing Rats. Molecular Neurobiology 56: 2760-2773, 2019. doi: 10.1007/s12035-018-1275-6. 

Seminotti, Bianca, Amaral AU, Ribeiro RT, Rodrigues MDN, Colín-González AL, Leipnitz G, Santamaría A, Wajner M. Oxidative Stress, Disrupted Energy Metabolism, and Altered Signaling Pathways in Glutaryl-CoA Dehydrogenase Knockout Mice: Potential Implications of Quinolinic Acid Toxicity in the Neuropathology of Glutaric Acidemia Type I. Molecular Neurobiology 53: 6459-6475, 2016. doi: 10.1007/s12035-015-9548-9. 

Seminotti, Bianca, Ribeiro RT, Amaral AU, da Rosa MS, Pereira CC, Leipnitz G, Koeller DM, Goodman S, Woontner M, Wajner M. Acute lysine overload provokes protein oxidative damage and reduction of antioxidant defenses in the brain of infant glutaryl-CoA dehydrogenase deficient mice: A role for oxidative stress in GA I neuropathology. Journal of the Neurological Sciences 344: 105-113, 2014. doi: 10.1016/j.jns.2014.06.034. 

Seminotti, Bianca, Amaral AU, da Rosa MS, Fernandes CG, Leipnitz G, Olivera-Bravo S, Barbeito L, Ribeiro CA, de Souza DO, Woontner M, Goodman SI, Koeller DM, Wajner M. Disruption of brain redox homeostasis in glutaryl-CoA dehydrogenase deficient mice treated with high dietary lysine supplementation. Molecular Genetics and Metabolism 108: 30-39, 2013. doi: 10.1016/j.ymgme.2012.11.001.

Academic and Research Interests

  • Inborn Errors of Metabolism
  • Fatty Acid Oxidation Disorders
  • Mitochondria