TY  - JOUR
AU  - Pagano, Giovanni
AU  - Talamanca, Annarita Aiello
AU  - Castello, Giuseppe
AU  - d'Ischia, Marco
AU  - Pallardo, Federico V.
AU  - Petrović, Sandra
AU  - Porto, Beatriz
AU  - Tiano, Luca
AU  - Zatterale, Adriana
PY  - 2013
UR  - https://vinar.vin.bg.ac.rs/handle/123456789/5454
AB  - Fanconi anemia (FA) is a rare genetic disease associated with deficiencies in DNA repair pathways. A body of literature points to a pro-oxidant state in FA patients, along with evidence for oxidative stress (OS) in the FA phenotype reported by in vitro, molecular, and animal studies. A highlight arises from the detection of mitochondrial dysfunction (MDF) in FA cell lines of complementation groups A, C, D2, and G. As yet lacking, in vivo studies should focus on FA-associated MDF, which may help in the understanding of the mitochondrial basis of OS detected in cells and body fluids from FA patients. Beyond the in vitro and animal databases, the available analytical devices may prompt the direct observation of metabolic and mitochondrial alterations in FA patients. These studies should evaluate a set of MDF-related endpoints, to be related to OS endpoints. The working hypothesis is raised that, parallel to OS, nitrosative stress might be another, so far unexplored, hallmark of the FA phenotype. The expected results may shed light on the FA pathogenesis and might provide grounds for pilot chemoprevention trials using mitochondrial nutrients. (C) 2013 Elsevier Inc. All rights reserved.
T2  - Free Radical Biology and Medicine
T1  - From clinical description, to in vitro and animal studies, and backward to patients: Oxidative stress and mitochondrial dysfunction in Fanconi anemia
VL  - 58
SP  - 118
EP  - 125
DO  - 10.1016/j.freeradbiomed.2013.01.015
ER  - 

@article{
author = "Pagano, Giovanni and Talamanca, Annarita Aiello and Castello, Giuseppe and d'Ischia, Marco and Pallardo, Federico V. and Petrović, Sandra and Porto, Beatriz and Tiano, Luca and Zatterale, Adriana",
year = "2013",
abstract = "Fanconi anemia (FA) is a rare genetic disease associated with deficiencies in DNA repair pathways. A body of literature points to a pro-oxidant state in FA patients, along with evidence for oxidative stress (OS) in the FA phenotype reported by in vitro, molecular, and animal studies. A highlight arises from the detection of mitochondrial dysfunction (MDF) in FA cell lines of complementation groups A, C, D2, and G. As yet lacking, in vivo studies should focus on FA-associated MDF, which may help in the understanding of the mitochondrial basis of OS detected in cells and body fluids from FA patients. Beyond the in vitro and animal databases, the available analytical devices may prompt the direct observation of metabolic and mitochondrial alterations in FA patients. These studies should evaluate a set of MDF-related endpoints, to be related to OS endpoints. The working hypothesis is raised that, parallel to OS, nitrosative stress might be another, so far unexplored, hallmark of the FA phenotype. The expected results may shed light on the FA pathogenesis and might provide grounds for pilot chemoprevention trials using mitochondrial nutrients. (C) 2013 Elsevier Inc. All rights reserved.",
journal = "Free Radical Biology and Medicine",
title = "From clinical description, to in vitro and animal studies, and backward to patients: Oxidative stress and mitochondrial dysfunction in Fanconi anemia",
volume = "58",
pages = "118-125",
doi = "10.1016/j.freeradbiomed.2013.01.015"
}

Pagano, G., Talamanca, A. A., Castello, G., d'Ischia, M., Pallardo, F. V., Petrović, S., Porto, B., Tiano, L.,& Zatterale, A.. (2013). From clinical description, to in vitro and animal studies, and backward to patients: Oxidative stress and mitochondrial dysfunction in Fanconi anemia. in Free Radical Biology and Medicine, 58, 118-125.
https://doi.org/10.1016/j.freeradbiomed.2013.01.015

Pagano G, Talamanca AA, Castello G, d'Ischia M, Pallardo FV, Petrović S, Porto B, Tiano L, Zatterale A. From clinical description, to in vitro and animal studies, and backward to patients: Oxidative stress and mitochondrial dysfunction in Fanconi anemia. in Free Radical Biology and Medicine. 2013;58:118-125.
doi:10.1016/j.freeradbiomed.2013.01.015 .

Pagano, Giovanni, Talamanca, Annarita Aiello, Castello, Giuseppe, d'Ischia, Marco, Pallardo, Federico V., Petrović, Sandra, Porto, Beatriz, Tiano, Luca, Zatterale, Adriana, "From clinical description, to in vitro and animal studies, and backward to patients: Oxidative stress and mitochondrial dysfunction in Fanconi anemia" in Free Radical Biology and Medicine, 58 (2013):118-125,
https://doi.org/10.1016/j.freeradbiomed.2013.01.015 . .

TY  - JOUR
AU  - Pagano, Giovanni
AU  - Aiello Talamanca, Annarita
AU  - Castello, Giuseppe
AU  - d'Ischia, Marco
AU  - Pallardo, Federico V.
AU  - Petrović, Sandra
AU  - Porto, Beatriz
AU  - Tiano, Luca
AU  - Zatterale, Adriana
PY  - 2013
UR  - https://vinar.vin.bg.ac.rs/handle/123456789/5582
AB  - Fanconi anaemia (FA) is a genetic cancer predisposition disorder associated with cytogenetic instability, bone marrow failure and a pleiotropic cellular phenotype, including low thresholds of responses to oxidative stress, cross-linking agents and selected cytokines. This study was aimed at defining the scope of abnormalities in gene expression using the publicly available FA Transcriptome Consortium (FTC) database (Gene Expression Omnibus, 2009 and publicly available as GSE16334). We evaluated the data set that included transcriptomal analyses on RNA obtained from low-density bone marrow cells (BMC) from 20 patients with FA and 11 healthy volunteers, by seeking to identify changes in expression of over 22000 genes, including a set of genes involved in: (i) bioenergetic pathways; (ii) antioxidant activities; (iii) response to stress and metal-chelating proteins; (iv) inflammation-related cytokines and (v) DNA repair. Ontological analysis of genes expressed at magnitudes of 1.5-fold or greater demonstrated significant suppression of genes in the categories of (i) energy metabolism; (ii) antioxidant activities; and (iii) stress and chelating proteins. Enhanced expression was found for 16 of 26 genes encoding inflammatory cytokines. A set of 20 of 21 transcripts for DNA repair activities were down-regulated; four of these transcripts related to type II topoisomerase. The data provide evidence for alterations in gene regulation of bioenergetic activities, redox-related activities, stress and metal-chelating proteins, and of some selected DNA repair activities in patients with FA.
T2  - European Journal of Haematology
T1  - Bone marrow cell transcripts from Fanconi anaemia patients reveal in vivo alterations in mitochondrial, redox and DNA repair pathways
VL  - 91
IS  - 2
SP  - 141
EP  - 151
DO  - 10.1111/ejh.12131
ER  - 

@article{
author = "Pagano, Giovanni and Aiello Talamanca, Annarita and Castello, Giuseppe and d'Ischia, Marco and Pallardo, Federico V. and Petrović, Sandra and Porto, Beatriz and Tiano, Luca and Zatterale, Adriana",
year = "2013",
abstract = "Fanconi anaemia (FA) is a genetic cancer predisposition disorder associated with cytogenetic instability, bone marrow failure and a pleiotropic cellular phenotype, including low thresholds of responses to oxidative stress, cross-linking agents and selected cytokines. This study was aimed at defining the scope of abnormalities in gene expression using the publicly available FA Transcriptome Consortium (FTC) database (Gene Expression Omnibus, 2009 and publicly available as GSE16334). We evaluated the data set that included transcriptomal analyses on RNA obtained from low-density bone marrow cells (BMC) from 20 patients with FA and 11 healthy volunteers, by seeking to identify changes in expression of over 22000 genes, including a set of genes involved in: (i) bioenergetic pathways; (ii) antioxidant activities; (iii) response to stress and metal-chelating proteins; (iv) inflammation-related cytokines and (v) DNA repair. Ontological analysis of genes expressed at magnitudes of 1.5-fold or greater demonstrated significant suppression of genes in the categories of (i) energy metabolism; (ii) antioxidant activities; and (iii) stress and chelating proteins. Enhanced expression was found for 16 of 26 genes encoding inflammatory cytokines. A set of 20 of 21 transcripts for DNA repair activities were down-regulated; four of these transcripts related to type II topoisomerase. The data provide evidence for alterations in gene regulation of bioenergetic activities, redox-related activities, stress and metal-chelating proteins, and of some selected DNA repair activities in patients with FA.",
journal = "European Journal of Haematology",
title = "Bone marrow cell transcripts from Fanconi anaemia patients reveal in vivo alterations in mitochondrial, redox and DNA repair pathways",
volume = "91",
number = "2",
pages = "141-151",
doi = "10.1111/ejh.12131"
}

Pagano, G., Aiello Talamanca, A., Castello, G., d'Ischia, M., Pallardo, F. V., Petrović, S., Porto, B., Tiano, L.,& Zatterale, A.. (2013). Bone marrow cell transcripts from Fanconi anaemia patients reveal in vivo alterations in mitochondrial, redox and DNA repair pathways. in European Journal of Haematology, 91(2), 141-151.
https://doi.org/10.1111/ejh.12131

Pagano G, Aiello Talamanca A, Castello G, d'Ischia M, Pallardo FV, Petrović S, Porto B, Tiano L, Zatterale A. Bone marrow cell transcripts from Fanconi anaemia patients reveal in vivo alterations in mitochondrial, redox and DNA repair pathways. in European Journal of Haematology. 2013;91(2):141-151.
doi:10.1111/ejh.12131 .

Pagano, Giovanni, Aiello Talamanca, Annarita, Castello, Giuseppe, d'Ischia, Marco, Pallardo, Federico V., Petrović, Sandra, Porto, Beatriz, Tiano, Luca, Zatterale, Adriana, "Bone marrow cell transcripts from Fanconi anaemia patients reveal in vivo alterations in mitochondrial, redox and DNA repair pathways" in European Journal of Haematology, 91, no. 2 (2013):141-151,
https://doi.org/10.1111/ejh.12131 . .