B
Bianca Seminotti
Researcher at University of Pittsburgh
Publications - 4
Citations - 118
Bianca Seminotti is an academic researcher from University of Pittsburgh. The author has contributed to research in topics: Mitochondrion & Respiratory chain. The author has an hindex of 4, co-authored 4 publications receiving 78 citations. Previous affiliations of Bianca Seminotti include Universidade Federal do Rio Grande do Sul.
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Journal ArticleDOI
Evaluation of mitochondrial bioenergetics, dynamics, endoplasmic reticulum-mitochondria crosstalk, and reactive oxygen species in fibroblasts from patients with complex I deficiency.
Guilhian Leipnitz,Guilhian Leipnitz,Al-Walid Mohsen,Anuradha Karunanidhi,Bianca Seminotti,Vera Roginskaya,Desiree M. Markantone,Mateus Grings,Mateus Grings,Stephanie J. Mihalik,Peter Wipf,Bennett Van Houten,Jerry Vockley +12 more
TL;DR: Treatment of ACAD9 deficient cells with JP4-039, a novel mitochondria-targeted reactive oxygen species, electron and radical scavenger, decreased superoxide level and increased basal and maximal respiratory rate is identified, identifying a potential therapeutic intervention opportunity in CI deficiency.
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Mitochondrial energetics is impaired in very long-chain acyl-CoA dehydrogenase deficiency and can be rescued by treatment with mitochondria-targeted electron scavengers.
Bianca Seminotti,Bianca Seminotti,Guilhian Leipnitz,Guilhian Leipnitz,Anuradha Karunanidhi,Catherine Kochersperger,Vera Roginskaya,Shrabani Basu,Yudong Wang,Peter Wipf,Bennett Van Houten,Al-Walid Mohsen,Jerry Vockley +12 more
TL;DR: The mitochondrially enriched electron and free radical scavengers JP4-039 and XJB-5-131 improved RC function and decreased ROS production significantly, suggesting that they are viable candidate compounds to further develop to treat VLCAD-deficient patients.
Journal ArticleDOI
ETHE1 and MOCS1 deficiencies: Disruption of mitochondrial bioenergetics, dynamics, redox homeostasis and endoplasmic reticulum-mitochondria crosstalk in patient fibroblasts
Mateus Grings,Bianca Seminotti,Bianca Seminotti,Anuradha Karunanidhi,Lina Ghaloul-Gonzalez,Al-Walid Mohsen,Peter Wipf,Johan Palmfeldt,Jerry Vockley,Guilhian Leipnitz,Guilhian Leipnitz +10 more
TL;DR: Data demonstrate that previously unrecognized broad disturbances of cellular function are involved in the pathophysiology of ETHE1 and MOCS1 deficiencies, and that reduction of mitochondrial superoxide by JP4-039 is a promising strategy for adjuvant therapy of these disorders.
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Reticular Dysgenesis and Mitochondriopathy Induced by Adenylate Kinase 2 Deficiency with Atypical Presentation.
Lina Ghaloul-Gonzalez,Al-Walid Mohsen,Anuradha Karunanidhi,Bianca Seminotti,Hey Chong,Suneeta Madan-Khetarpal,Jessica Sebastian,Catherine Walsh Vockley,Miguel Reyes-Múgica,Mark Vander Lugt,Jerry Vockley +10 more
TL;DR: It is demonstrated that reticular dysgenesis should be considered in Amish individuals presenting with immune deficiency and pathophysiological aspects of AK2 deficiency not previously reported are described.