المؤلفون: Corrà, Samantha, Cerutti, Raffaele, Balmaceda, Valeria, Viscomi, Carlo, Zeviani, Massimo

المساهمون: Telethon Foundation, Fondation NRJ pour les Neurosciences - Institute de France Grant, Associazione Luigi Comini Onlus, AFM – Telethon

المصدر: Brain ; volume 145, issue 10, page 3405-3414 ; ISSN 0006-8950 1460-2156

الاتاحة: https://doi.org/10.1093/brain/awac182
https://academic.oup.com/brain/article-pdf/145/10/3405/46598522/awac182.pdf

المؤلفون: Silva-Pinheiro, Pedro, Pardo-Hernández, Carlos, Reyes, Aurelio, Tilokani, Lisa, Mishra, Anup, Cerutti, Raffaele, Li, Shuaifeng, Rozsivalova, Dieu-Hien, Valenzuela, Sebastian, Dogan, Sukru A, Peter, Bradley, Fernández-Silva, Patricio, Trifunovic, Aleksandra, Prudent, Julien, Minczuk, Michal, Bindoff, Laurence, Macao, Bertil, Zeviani, Massimo, Falkenberg, Maria, Viscomi, Carlo

المساهمون: Silva-Pinheiro, Pedro, Pardo-Hernández, Carlo, Reyes, Aurelio, Tilokani, Lisa, Mishra, Anup, Cerutti, Raffaele, Li, Shuaifeng, Rozsivalova, Dieu-Hien, Valenzuela, Sebastian, Dogan, Sukru A, Peter, Bradley, Fernández-Silva, Patricio, Trifunovic, Aleksandra, Prudent, Julien, Minczuk, Michal, Bindoff, Laurence, Macao, Bertil, Zeviani, Massimo, Falkenberg, Maria, Viscomi, Carlo

Relation: info:eu-repo/semantics/altIdentifier/pmid/33956154; info:eu-repo/semantics/altIdentifier/wos/WOS:000658215500037; volume:49; issue:9; firstpage:5230-5248; lastpage:5248; journal:NUCLEIC ACIDS RESEARCH; http://hdl.handle.net/11577/3391683; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85107086902

الاتاحة: http://hdl.handle.net/11577/3391683
https://doi.org/10.1093/nar/gkab282

المؤلفون: CERUTTI, RAFFAELE

المساهمون: Cerutti, Raffaele, PEGORARO, ELENA

مصطلحات موضوعية: Settore BIO/11 - Biologia Molecolare

Relation: https://hdl.handle.net/11577/3520424

الاتاحة: https://hdl.handle.net/11577/3520424

المؤلفون: Brunetti, Dario, Torsvik, Janniche, Dallabona, Cristina, Teixeira, Pedro, Sztromwasser, Pawel, Fernandez-Vizarra, Erika, Cerutti, Raffaele, Reyes, Aurelio, Preziuso, Carmela, D'Amati, Giulia, Baruffini, Enrico, Goffrini, Paola, Viscomi, Carlo, Ferrero, Ileana, Boman, Helge, Telstad, Wenche, Johansson, Stefan, Glaser, Elzbieta, Knappskog, Per M., Zeviani, Massimo, Bindoff, Laurence A.

المصدر: EMBO Molecular Medicine. 8(3):176-190

مصطلحات موضوعية: amyloid beta, mitochondrial targeting sequence, mitochondrial disease, neurodegeneration, pitrilysin 1

وصف الملف: print

URL الوصول: https://urn.kb.se/resolve?urn=urn:nbn:se:su:diva-129217
https://doi.org/10.15252/emmm.201505894

المؤلفون: Silva-Pinheiro, Pedro, Pardo-Hernández, Carlos, Reyes, Aurelio, Tilokani, Lisa, Mishra, Anup, Cerutti, Raffaele, Li, Shuaifeng, Rozsivalova, Dieu-Hien, Valenzuela, Sebastian, Dogan, Sukru A, Peter, Bradley, Fernández-Silva, Patricio, Trifunovic, Aleksandra, Prudent, Julien, Minczuk, Michal, Bindoff, Laurence, Macao, Bertil, Zeviani, Massimo, Falkenberg, Maria, Viscomi, Carlo

المصدر: Nucleic Acids Research ; volume 49, issue 18, page 10803-10803 ; ISSN 0305-1048 1362-4962

مصطلحات موضوعية: Genetics

الاتاحة: http://dx.doi.org/10.1093/nar/gkab837
http://academic.oup.com/nar/article-pdf/49/18/10803/40538526/gkab837.pdf

المؤلفون: Luna-Sanchez, Marta, Benincá, Cristiane, Cerutti, Raffaele, Brea-Calvo, Gloria, Yeates, Anna, Scorrano, Luca, Zeviani, Massimo, Viscomi, Carlo

المساهمون: Telethon Italia, European Research Council, Medical Research Council (UK), European Commission

Relation: #PLACEHOLDER_PARENT_METADATA_VALUE#; info:eu-repo/grantAgreement/EC/FP7/322424; Publisher's version; https://doi.org/10.1016/j.ymthe.2020.06.010; Sí; Molecular Therapy 28(8): 1918-1930 (2020); http://hdl.handle.net/10261/222572; http://dx.doi.org/10.13039/501100000780; http://dx.doi.org/10.13039/501100000781; http://dx.doi.org/10.13039/501100000265

الاتاحة: http://hdl.handle.net/10261/222572
https://doi.org/10.1016/j.ymthe.2020.06.010
https://doi.org/10.13039/501100000780
https://doi.org/10.13039/501100000781
https://doi.org/10.13039/501100000265

المؤلفون: Signes, Alba, Cerutti, Raffaele, Dickson, Anna S, Benincá, Cristiane, Hinchy, Elizabeth C, Ghezzi, Daniele, Carrozzo, Rosalba, Bertini, Enrico, Murphy, Michael P, Nathan, James A, Viscomi, Carlo, Fernandez-Vizarra, Erika, Zeviani, Massimo

المساهمون: A. Signe, R. Cerutti, A.S. Dickson, C. Benincá, E.C. Hinchy, D. Ghezzi, R. Carrozzo, E. Bertini, M.P. Murphy, J.A. Nathan, C. Viscomi, E. Fernandez-Vizarra, M. Zeviani

مصطلحات موضوعية: APOPT1‐COA8, cytochrome c oxidase, mitochondrial encephalopathy, proteasome–ubiquitin system, reactive oxygen species, Settore MED/03 - Genetica Medica, Settore BIO/11 - Biologia Molecolare

Relation: info:eu-repo/semantics/altIdentifier/pmid/30552096; info:eu-repo/semantics/altIdentifier/wos/WOS:000455807100008; volume:11; issue:1; firstpage:1; lastpage:21; numberofpages:21; journal:EMBO MOLECULAR MEDICINE; http://hdl.handle.net/2434/612747; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85058464983

الاتاحة: http://hdl.handle.net/2434/612747
https://doi.org/10.15252/emmm.201809582

المؤلفون: Dogan, Sukru Anil, Cerutti, Raffaele, Benincá, Cristiane, Brea-Calvo, Gloria, Jacobs, Howard Trevor, Zeviani, Massimo, Szibor, Marten, Viscomi, Carlo

المساهمون: Institute of Biotechnology

مصطلحات موضوعية: mitochondrial biogenesis, alternative oxidase, ROS, redox signaling, autophagy, mitochondrial disease, antioxidant, stress responses, satellite cells, Biochemistry, cell and molecular biology

وصف الملف: application/pdf

Relation: Dogan , S A , Cerutti , R , Benincá , C , Brea-Calvo , G , Jacobs , H T , Zeviani , M , Szibor , M & Viscomi , C 2018 , ' Perturbed Redox Signaling Exacerbates a Mitochondrial Myopathy ' , Cell Metabolism , vol. 28 , no. 5 , pp. 764-+ . https://doi.org/10.1016/j.cmet.2018.07.012; RIS: urn:F7620609A1C62FE30FFBC00FB5B0771E; http://hdl.handle.net/10138/288729; 0173569a-78ff-4521-9474-481560e9c0a4; 85054720281; 000449440000011

الاتاحة: http://hdl.handle.net/10138/288729

المؤلفون: Bottani, Emanuela, Cerutti, Raffaele, Harbour, Michael E, Ravaglia, Sabrina, Dogan, Sukru Anil, Giordano, Carla, Fearnley, Ian M, D'Amati, Giulia, Viscomi, Carlo, Fernandez-Vizarra, Erika, Zeviani, Massimo

المساهمون: Bottani, Emanuela, Cerutti, Raffaele, Harbour, Michael E, Ravaglia, Sabrina, Dogan, Sukru Anil, Giordano, Carla, Fearnley, Ian M, D'Amati, Giulia, Viscomi, Carlo, Fernandez-Vizarra, Erika, Zeviani, Massimo

مصطلحات موضوعية: Rieske protein, TTC19, UQCRFS1, complex III deficiency, mitochondrial complex III, mitochondrial disease, mitochondrial quality control, mitochondrial respiratory chain, mouse model

وصف الملف: ELETTRONICO

Relation: info:eu-repo/semantics/altIdentifier/pmid/28673544; info:eu-repo/semantics/altIdentifier/wos/WOS:000404897300010; volume:67; issue:1; firstpage:96; lastpage:105; numberofpages:10; journal:MOLECULAR CELL; http://hdl.handle.net/11562/1030665; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85021312011; https://www.sciencedirect.com/science/article/pii/S1097276517304033

الاتاحة: http://hdl.handle.net/11562/1030665
https://doi.org/10.1016/j.molcel.2017.06.001
https://www.sciencedirect.com/science/article/pii/S1097276517304033

المؤلفون: Viscomi, CF, Qualdalti, Corinne, Brunetti, Dario, Cerutti, Raffaele, Johnson, Mark, Bottani, Emanuela, Fernandez-Vizarra, Erika, Robinson, Alan, Zeviani, Massimo

مصطلحات موضوعية: Genome editing, Leigh syndrome, Mitochondrial disease, Pig, SURF1 KO, Animals, Genetically Modified, Newborn, Behavior, Animal, CRISPR-Cas Systems, Cells, Cultured, Central Nervous System, Disease Models, Down-Regulation, Electron Transport Complex IV, Female, Fibroblasts, Gene Editing, Gene Knockout Techniques, Humans, Jejunum, Leigh Disease, Male, Membrane Proteins, Mitochondria, Mitochondrial Proteins, Muscle, Skeletal

وصف الملف: application/pdf; application/vnd.openxmlformats-officedocument.wordprocessingml.document

Relation: https://www.repository.cam.ac.uk/handle/1810/277288

الاتاحة: https://www.repository.cam.ac.uk/handle/1810/277288
https://doi.org/10.17863/CAM.24572

المؤلفون: Civiletto, Gabriele, Dogan, Sukru Anil, Cerutti, Raffaele, Fagiolari, Gigliola, Moggio, Maurizio, Lamperti, Costanza, Benincá, Cristiane, Viscomi, Carlo, Zeviani, Massimo

مصطلحات موضوعية: autophagy, lysosomal biogenesis, mTORC1, mitochondrial disease, rapamycin, Animals, Basic Helix-Loop-Helix Leucine Zipper Transcription Factors, Electron Transport Complex IV, Lysosomes, Mice, Inbred C57BL, Knockout, Mitochondria, Mitochondrial Myopathies, Motor Activity, Muscles, Organelle Biogenesis, Phenotype, Rilmenidine, Sirolimus, TOR Serine-Threonine Kinases

وصف الملف: Print; application/pdf

Relation: https://www.repository.cam.ac.uk/handle/1810/287129

الاتاحة: https://www.repository.cam.ac.uk/handle/1810/287129
https://doi.org/10.17863/CAM.34438

المؤلفون: Dogan, Sukru Anil, Cerutti, Raffaele, Benincá, Cristiane, Brea-Calvo, Gloria, Jacobs, Howard Trevor, Zeviani, Massimo, Szibor, Marten, Viscomi, Carlo

مصطلحات موضوعية: ROS, alternative oxidase, antioxidant, autophagy, mitochondrial biogenesis, mitochondrial disease, redox signaling, satellite cells, stress responses, Animals, Female, Male, Mice, Inbred C57BL, Knockout, Mitochondrial Myopathies, Mitochondrial Proteins, Muscle, Skeletal, Organelle Biogenesis, Oxidation-Reduction, Oxidoreductases, Plant Proteins, Reactive Oxygen Species, Signal Transduction

وصف الملف: Print-Electronic; application/pdf

Relation: https://www.repository.cam.ac.uk/handle/1810/279931

الاتاحة: https://www.repository.cam.ac.uk/handle/1810/279931
https://doi.org/10.17863/CAM.27299

المؤلفون: Viscomi, Carlo, Dogan, Anil, Cerutti, Raffaele, Civiletto, Gabriele, Beninca’, Cristiane, Fagiolari, Gigliola, Moggio, Maurizio, Jacobs, Howy, Szibor, Marten, Zeviani, Massimo

المصدر: Biochimica et Biophysica Acta (BBA) - Bioenergetics ; volume 1859, page e26 ; ISSN 0005-2728

الاتاحة: http://dx.doi.org/10.1016/j.bbabio.2018.09.082
https://api.elsevier.com/content/article/PII:S0005272818303517?httpAccept=text/xml
https://api.elsevier.com/content/article/PII:S0005272818303517?httpAccept=text/plain

المؤلفون: Civiletto, Gabriele, Varanita, Tatiana, Cerutti, Raffaele, Gorletta, Tatiana, Barbaro, Serena, Marchet, Silvia, Lamperti, Costanza, Viscomi, Carlo, Scorrano, Luca, Zeviani, Massimo

المصدر: Cell Metabolism ; volume 21, issue 6, page 845-854 ; ISSN 1550-4131

الاتاحة: http://dx.doi.org/10.1016/j.cmet.2015.04.016
https://api.elsevier.com/content/article/PII:S1550413115001710?httpAccept=text/xml
https://api.elsevier.com/content/article/PII:S1550413115001710?httpAccept=text/plain

المؤلفون: Silva-Pinheiro, Pedro, Pardo-Hernandez, Carlos, Reyes, Aurelio, Tilokani, Lisa, Mishra, Anup, Cerutti, Raffaele, Li, Shuaifeng, Rozsivalova, Dieu-Hien, Valenzuela, Sebastian, Dogan, Sukru A., Peter, Bradley, Fernandez-Silva, Patricio, Trifunovic, Aleksandra, Prudent, Julien, Minczuk, Michal, Bindoff, Laurence, Macao, Bertil, Zeviani, Massimo, Falkenberg, Maria, Viscomi, Carlo

مصطلحات موضوعية: ddc:no

Relation: Silva-Pinheiro, Pedro orcid:0000-0002-0872-5749 , Pardo-Hernandez, Carlos, Reyes, Aurelio orcid:0000-0003-2876-2202 , Tilokani, Lisa, Mishra, Anup, Cerutti, Raffaele, Li, Shuaifeng, Rozsivalova, Dieu-Hien, Valenzuela, Sebastian orcid:0000-0001-5153-8431 , Dogan, Sukru A., Peter, Bradley, Fernandez-Silva, Patricio, Trifunovic, Aleksandra, Prudent, Julien, Minczuk, Michal orcid:0000-0001-8242-1420 , Bindoff, Laurence, Macao, Bertil, Zeviani, Massimo, Falkenberg, Maria and Viscomi, Carlo orcid:0000-0001-6050-0566 (2021). DNA polymerase gamma mutations that impair holoenzyme stability cause catalytic subunit depletion (vol 49, pg 5230, 2021). Nucleic Acids Res., 49 (18). S. 10803 - 10804. OXFORD: OXFORD UNIV PRESS. ISSN 1362-4962

الاتاحة: https://kups.ub.uni-koeln.de/60075/

المؤلفون: Cerutti, Raffaele, Pirinen, Eija, Lamperti, Costanza, Marchet, Silvia, Sauve, Anthony A., Li, Wei, Leoni, Valerio, Schon, Eric A., Dantzer, Françoise, Auwerx, Johan, Viscomi, Carlo, Zeviani, Massimo

المصدر: Cell Metabolism ; volume 19, issue 6, page 1042-1049 ; ISSN 1550-4131

الاتاحة: http://dx.doi.org/10.1016/j.cmet.2014.04.001
https://api.elsevier.com/content/article/PII:S1550413114001648?httpAccept=text/xml
https://api.elsevier.com/content/article/PII:S1550413114001648?httpAccept=text/plain

المؤلفون: Corrà, Samantha, Cerutti, Raffaele, Balmaceda, Valeria, Viscomi, Carlo, Zeviani, Massimo

المصدر: Brain: A Journal of Neurology; Oct2022, Vol. 145 Issue 10, p3405-3414, 10p

مصطلحات موضوعية: MAMMAL metabolism, PROTEIN metabolism, ENERGY metabolism, BIOLOGICAL models, PROTEINS, VIRUSES, LEIGH disease, ANIMALS, MICE

المؤلفون: Viscomi, Carlo, Bottani, Emanuela, Civiletto, Gabriele, Cerutti, Raffaele, Moggio, Maurizio, Fagiolari, Gigliola, Schon, Eric A., Lamperti, Costanza, Zeviani, Massimo

المصدر: Cell Metabolism ; volume 14, issue 1, page 80-90 ; ISSN 1550-4131

الاتاحة: http://dx.doi.org/10.1016/j.cmet.2011.04.011
https://api.elsevier.com/content/article/PII:S1550413111002117?httpAccept=text/xml
https://api.elsevier.com/content/article/PII:S1550413111002117?httpAccept=text/plain

المؤلفون: Balconi, Emanuela, Pennati, Andrea, Crobu, Danila, Pandini, Vittorio, Cerutti, Raffaele, Zanetti, Giuliana, Aliverti, Alessandro

المصدر: The FEBS Journal ; volume 276, issue 14, page 3825-3836 ; ISSN 1742-464X 1742-4658

الاتاحة: http://dx.doi.org/10.1111/j.1742-4658.2009.07100.x
https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1111%2Fj.1742-4658.2009.07100.x
https://febs.onlinelibrary.wiley.com/doi/pdf/10.1111/j.1742-4658.2009.07100.x

المؤلفون: Telethon Italia, European Research Council, Medical Research Council (UK), European Commission, Luna-Sanchez, Marta, Benincá, Cristiane, Cerutti, Raffaele, Brea-Calvo, Gloria, Yeates, Anna, Scorrano, Luca, Zeviani, Massimo, Viscomi, Carlo

مصطلحات الفهرس: artículo

URL: http://hdl.handle.net/10261/222572
https://doi.org/10.1016/j.ymthe.2020.06.010
Publisher's version
https://doi.org/10.1016/j.ymthe.2020.06.010
Sí
info:eu-repo/grantAgreement/EC/FP7/322424