Defects in the Maturation of Mitochondrial Iron–Sulfur Proteins: Biophysical Investigation of the MMDS3 Causing Gly104Cys Variant of IBA57

Autor: Beatrice Bargagna, Tommaso Staderini, Steven H. Lang, Lucia Banci, Francesca Camponeschi

Publikováno v: International Journal of Molecular Sciences, Vol 25, Iss 19, p 10466 (2024)

Multiple mitochondrial dysfunctions syndrome type 3 (MMDS3) is a rare autosomal recessive mitochondrial leukoencephalopathy caused by biallelic pathogenic variants in the IBA57 gene. The gene protein product, IBA57, has an unknown role in iron–sulf

Externí odkaz: https://doaj.org/article/a3607a8384184f4087228fb202fc38b7

Copper Binding and Redox Activity of α-Synuclein in Membrane-Like Environment

Autor: Chiara Bacchella, Francesca Camponeschi, Paulina Kolkowska, Arian Kola, Isabella Tessari, Maria Camilla Baratto, Marco Bisaglia, Enrico Monzani, Luigi Bubacco, Stefano Mangani, Luigi Casella, Simone Dell’Acqua, Daniela Valensin

Publikováno v: Biomolecules, Vol 13, Iss 2, p 287 (2023)

α-Synuclein (αSyn) constitutes the main protein component of Lewy bodies, which are the pathologic hallmark in Parkinson’s disease. αSyn is unstructured in solution but the interaction of αSyn with lipid membrane modulates its conformation by i

Externí odkaz: https://doaj.org/article/ddf2649895b1479687fce0f3f6237b92

Molecular Basis of Rare Diseases Associated to the Maturation of Mitochondrial [4Fe-4S]-Containing Proteins

Autor: Francesca Camponeschi, Simone Ciofi-Baffoni, Vito Calderone, Lucia Banci

Publikováno v: Biomolecules, Vol 12, Iss 7, p 1009 (2022)

The importance of mitochondria in mammalian cells is widely known. Several biochemical reactions and pathways take place within mitochondria: among them, there are those involving the biogenesis of the iron–sulfur (Fe-S) clusters. The latter are ev

Externí odkaz: https://doaj.org/article/66275a09e5ad41f9a486720d00a80761

The Intriguing mitoNEET: Functional and Spectroscopic Properties of a Unique [2Fe-2S] Cluster Coordination Geometry

Autor: LUCIA BANCI, Francesca Camponeschi, Mario Piccioli

Publikováno v: Molecules

Despite the number of cellular and pathological mitoNEET-related processes, very few details are known about the mechanism of action of the protein. The recently discovered existence of a link between NEET proteins and cancer pave the way to consider

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::bb424c91042d935f9d65d18fd56608dd
https://pubmed.ncbi.nlm.nih.gov/36500311

In Cellulo Mössbauer and EPR Studies Bring New Evidence to the Long‐Standing Debate on Iron–Sulfur Cluster Binding in Human Anamorsin

Autor: Geneviève Blondin, Martin Clémancey, Lucia Banci, Francesca Camponeschi, Simone Ciofi-Baffoni, Sara Matteucci

Publikováno v: Angewandte Chemie International Edition
Angewandte Chemie International Edition, Wiley-VCH Verlag, 2021, 60 (27), pp.14841-14845. ⟨10.1002/anie.202102910⟩
Angewandte Chemie (International Ed. in English)
Angewandte Chemie International Edition, 2021, 60 (27), pp.14841-14845. ⟨10.1002/anie.202102910⟩

Human anamorsin is an iron–sulfur (Fe–S)‐cluster‐binding protein acting as an electron donor in the early steps of cytosolic iron–sulfur protein biogenesis. Human anamorsin belongs to the eukaryotic CIAPIN1 protein family and contains two h

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5bcc73a995becac224d4941e2dcc8298
https://doi.org/10.1002/anie.202102910