Publications by authors named "Beatrice Bargagna"
Article Synopsis
- - Multiple mitochondrial dysfunctions syndrome type 3 (MMDS3) is a rare genetic disorder caused by mutations in the IBA57 gene, which plays a role in the maturation of specific mitochondrial proteins.
- - The pathogenic variant p.Gly104Cys in the IBA57 gene is linked to severe forms of MMDS3 and leads to issues with mitochondrial respiratory complexes and enzyme functionality in affected individuals.
- - Research techniques like NMR, circular dichroism, and fluorescence spectroscopy revealed that while the p.Gly104Cys variant doesn't affect the formation of IBA57 complexes, it significantly destabilizes the IBA57 protein, contributing to the serious symptoms of MMDS3.
Multiple mitochondrial dysfunctions syndrome type 2 with hyperglycinemia (MMDS2) is a severe disorder of mitochondrial energy metabolism, associated with biallelic mutations in the gene encoding for BOLA3, a protein with a not yet completely understood role in iron-sulfur (Fe-S) cluster biogenesis, but essential for the maturation of mitochondrial [4Fe-4S] proteins. To better understand the role of BOLA3 in MMDS2, we have investigated the impact of the p.His96Arg (c.
View Article and Find Full Text PDF[4Fe-4S] cluster assembly in human cytosol requires both a [2Fe-2S] cluster chaperone being able to donate two [2Fe-2S] clusters and an electron donor providing two electrons to reductively couple the two [2Fe-2S] clusters into a [4Fe-4S] cluster. The mechanism through which the cytosolic [4Fe-4S] cluster assembly works is still not defined. Here, we show that a hetero-tetrameric complex formed by two molecules of cluster-reduced [2Fe-2S] -anamorsin and one molecule of dimeric cluster-oxidized [2Fe-2S] -GLRX3 orchestrates the assembly of a [4Fe-4S] cluster on the N-terminal cluster binding site of the cytosolic protein NUBP1.
View Article and Find Full Text PDFArticle Synopsis
- Epidemiological studies indicate that a diet high in fruits and vegetables can lower the risk of neurodegenerative diseases.
- Resveratrol (Res) is known for its neuroprotective benefits, but its effectiveness is limited due to quick metabolism and low bioavailability, whereas pterostibene (Ptb) has better properties in these areas.
- The study introduces two fluorescent derivatives of Ptb for further research, detailing their synthesis, optical and structural characterization, and potential cytotoxic effects on different cell lines.*
Article Synopsis
- - Alzheimer's disease (AD) is a complex neurodegenerative disorder that causes progressive neuron loss, and its causes and progression mechanisms are still not fully understood.
- - Currently, there are limited symptomatic treatments for AD, highlighting the need for developing new therapies that can address multiple pathological aspects of the disease.
- - This study presents five newly synthesized ferulic acid-based hybrid compounds that show potential as anti-AD agents by maintaining the beneficial properties of ferulic acid while effectively inhibiting toxic Aβ aggregation, and they have favorable pharmacokinetic profiles for absorption.