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Julia Gabrieli Bender

ORCID: 0000-0002-2309-2417
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About
Contact & Profiles
A5043670633
Research Areas
  • Metabolism and Genetic Disorders
  • Peroxisome Proliferator-Activated Receptors
  • Amino Acid Enzymes and Metabolism
  • Mitochondrial Function and Pathology
  • Metabolomics and Mass Spectrometry Studies

Universidade Federal do Rio Grande do Sul
 2023-2024

 Oxidative Stress Associated With Increased Reactive Nitrogen Species Generation in the Liver and Kidney Caused by a Major Metabolite Accumulating in Tyrosinemia Type 1
OPENALEX - Publications 
Julia Gabrieli Bender Rafael Teixeira Ribeiro Ângela Beatris Zemniaçak Rafael Palavro Rafael Aguiar Marschner and 5 more Simone Magagnin Wajner Ediandra Tissot Castro Guilhian Leipnitz Moaçir Wajner Alexandre Umpierrez Amaral

ABSTRACT Tyrosinemia type 1 (TT1) is caused by fumarylacetoacetate hydrolase activity deficiency, resulting in tissue accumulation of upstream metabolites, including succinylacetone (SA), the pathognomonic compound this disease. Since pathogenesis liver and kidney damage observed TT1‐affected patients practically unknown, study assessed effects SA on important biomarkers redox homeostasis adolescent rats, as well hepatic (HepG2) renal (HEK‐293) cultured cells. significantly increased nitrate...

10.1002/cbf.70010 article EN Cell Biochemistry and Function 2024-10-27
 Disruption of mitochondrial bioenergetics and calcium homeostasis by phytanic acid in the heart: Potential relevance for the cardiomyopathy in Refsum disease
OPENALEX - Publications 
Ângela Beatris Zemniaçak Ana Cristina Roginski Rafael Teixeira Ribeiro Julia Gabrieli Bender Rafael Aguiar Marschner and 3 more Simone Magagnin Wajner Moaçir Wajner Alexandre Umpierrez Amaral

10.1016/j.bbabio.2023.148961 article EN publisher-specific-oa Biochimica et Biophysica Acta (BBA) - Bioenergetics 2023-02-20
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