Exploring the Ability of LARS2 Carboxy-Terminal Domain in Rescuing the MELAS Phenotype

Autor: Francesco Capriglia, Francesca Rizzo, Giuseppe Petrosillo, Veronica Morea, Giulia d’Amati, Palmiro Cantatore, Marina Roberti, Paola Loguercio Polosa, Francesco Bruni

Publikováno v: Life, Vol 11, Iss 7, p 674 (2021)

The m.3243A>G mutation within the mitochondrial mt-tRNALeu(UUR) gene is the most prevalent variant linked to mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome. This pathogenic mutation causes severe impairmen

Externí odkaz: https://doaj.org/article/abe9ed0abae04b0196ecea47b2475768

Exploring the Ability of LARS2 Carboxy-Terminal Domain in Rescuing the MELAS Phenotype

Autor: Giulia d'Amati, Palmiro Cantatore, Marina Roberti, Veronica Morea, Francesco Bruni, Paola Loguercio Polosa, Francesca Maria Rizzo, Giuseppe Petrosillo, Francesco Capriglia

Publikováno v: Life, Vol 11, Iss 674, p 674 (2021)
Life
Volume 11
Issue 7
Life (Basel) 11 (2021). doi:10.3390/life11070674
info:cnr-pdr/source/autori:Capriglia F.; Rizzo F.; Petrosillo G.; Morea V.; D'amati G.; Cantatore P.; Roberti M.; Loguercio Polosa P.; Bruni F./titolo:Exploring the ability of lars2 carboxy-terminal domain in rescuing the melas phenotype/doi:10.3390%2Flife11070674/rivista:Life (Basel)/anno:2021/pagina_da:/pagina_a:/intervallo_pagine:/volume:11

The m.3243A>
G mutation within the mitochondrial mt-tRNALeu(UUR) gene is the most prevalent variant linked to mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome. This pathogenic mutation causes severe i

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8b0b044db5db1f1c8173534be8f89fdd
https://www.mdpi.com/2075-1729/11/7/674

Fine-tuning of the respiratory complexes stability and supercomplexes assembly in cells defective of complex III

Autor: Letizia Scandiffio, Claudia Zanna, Michela Rugolo, Jessica Fiori, Stefan Steimle, Fevzi Daldal, Serena J. Aleo, Marina Roberti, Aldo Roda, Paola Loguercio Polosa, Anna Ghelli, Valerio Carelli, Emanuele Porru, Concetta Valentina Tropeano

Publikováno v: Biochim Biophys Acta Bioenerg

The respiratory complexes are organized in supramolecular assemblies called supercomplexes thought to optimize cellular metabolism under physiological and pathological conditions. In this study, we used genetically and biochemically well characterize

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6a188689001c1f1bfd2404aac9948a74
https://pubmed.ncbi.nlm.nih.gov/31825807

MTERF factors: a multifunction protein family

Autor: Paola Loguercio Polosa, Maria Nicola Gadaleta, Stefania Deceglie, Palmiro Cantatore, Francesco Bruni, Marina Roberti

Publikováno v: Biomolecular Concepts, Vol 1, Iss 2, Pp 215-224 (2010)

The MTERF family is a large protein family, identified in metazoans and plants, which consists of four subfamilies, MTERF1, 2, 3 and 4. Mitochondrial localisation was predicted for the vast majority of MTERF family members and demonstrated for the ch

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::fa9643c1fc53c1d535268e5e3c1b82ee
https://doi.org/10.1515/bmc.2010.015

Cigarette toxicity triggers Leber's hereditary optic neuropathy by affecting mtDNA copy number, oxidative phosphorylation and ROS detoxification pathways

Autor: Pio D'Adamo, Flavio Fracasso, Luca Giordano, Giuseppe Petrosillo, Palmiro Cantatore, Anna Ghelli, Dino Parente, S Ciaravolo, Valerio Carelli, V. Del Dotto, Alessandra Maresca, Stefania Deceglie, Marina Roberti, C. La Morgia, P. Loguercio Polosa, Adriana Berezovsky, Carla Giordano, Luisa Iommarini, Solange Rios Salomão, Maria Lucia Valentino, M Cappellari, Rubens N. Belfort, Alfredo A. Sadun

Publikováno v: Cell Death & Disease
Cell death & disease, vol 6, iss 12
Cell death and disease 6 (2015). doi:10.1038/cddis.2015.364
info:cnr-pdr/source/autori:Giordano, L.; Deceglie, S.; d'Adamo, P.; Valentino, Ml; La Morgia, C.; Fracasso, F.; Roberti, M.; Cappellari, M.; Petrosillo, G.; Ciaravolo, S.; Parente, D.; Giordano, C.; Maresca, A.; Iommarini, L.; Del Dotto, V.; Ghelli, A. M.; Salomao, S. R.; Berezovsky, A.; Belfort, R., Jr.; Sadun, A. A.; Carelli, V.; Polosa, P. Loguercio; Cantatore, P./titolo:Cigarette toxicity triggers Leber's hereditary optic neuropathy by affecting mtDNA copy number, oxidative phosphorylation and ROS detoxification pathways/doi:10.1038%2Fcddis.2015.364/rivista:Cell death and disease/anno:2015/pagina_da:/pagina_a:/intervallo_pagine:/volume:6

Leber’s hereditary optic neuropathy (LHON), the most frequent mitochondrial disease, is associated with mitochondrial DNA (mtDNA) point mutations affecting Complex I subunits, usually homoplasmic. This blinding disorder is characterized by incomple

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c673f612ba4234179f5e17b0dac798e7
https://hdl.handle.net/11368/2851603