Commentary: The m-AAA Protease Associated with Neurodegeneration Limits MCU Activity in Mitochondria

被引：4

作者：

Bernardi, Paolo ^{[1
]}

Forte, Michael ^{[2
]}

机构：

[1] Univ Padua, Dept Biomed Sci, Padua, Italy

[2] Oregon Hlth & Sci Univ, Vollum Inst, L474, Portland, OR 97201 USA

来源：

FRONTIERS IN PHYSIOLOGY | 2016年 / 7卷

关键词：

mitochondria; proteases; calcium uniporter; permeability transition pore; neurodegenerative diseases; PARAPLEGIN; AFG3L2; IMPAIRMENT; MUTATIONS; COMPONENT; ATAXIA;

D O I：

10.3389/fphys.2016.00583

中图分类号：

Q4 [生理学];

学科分类号：

071003 [生理学];

摘要：

引用

页数：3

共 21 条

[1]

AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival [J].

Almajan, Eva R. ;

Richter, Ricarda ;

Paeger, Lars ;

Martinelli, Paola ;

Barth, Esther ;

Decker, Thorsten ;

Larsson, Nils-Goeran ;

Kloppenburg, Peter ;

Langer, Thomas ;

Rugarli, Elena I. .

JOURNAL OF CLINICAL INVESTIGATION, 2012, 122 (11) :4048-4058

[2]

Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia [J].

Atorino, L ;

Silvestri, L ;

Koppen, M ;

Cassina, L ;

Ballabio, A ;

Marconi, R ;

Langer, T ;

Casari, G .

JOURNAL OF CELL BIOLOGY, 2003, 163 (04) :777-787

[3]

Identification and characterization of AFG3L2, a novel paraplegin-related gene [J].

Banfi, S ;

Bassi, MT ;

Andolfi, G ;

Marchitiello, A ;

Zanotta, S ;

Ballabio, A ;

Casari, G ;

Franco, B .

GENOMICS, 1999, 59 (01) :51-58

[4]

Integrative genomics identifies MCU as an essential component of the mitochondrial calcium uniporter [J].

Baughman, Joshua M. ;

Perocchi, Fabiana ;

Girgis, Hany S. ;

Plovanich, Molly ;

Belcher-Timme, Casey A. ;

Sancak, Yasemin ;

Bao, X. Robert ;

Strittmatter, Laura ;

Goldberger, Olga ;

Bogorad, Roman L. ;

Koteliansky, Victor ;

Mootha, Vamsi K. .

NATURE, 2011, 476 (7360) :341-U111

[5]

Commentary: SPG7 is an essential and conserved component of the mitochondrial permeability transition pore [J].

Bernardi, Paolo ;

Forte, Michael .

FRONTIERS IN PHYSIOLOGY, 2015, 6

[6]

THE MITOCHONDRIAL PERMEABILITY TRANSITION PORE: CHANNEL FORMATION BY F-ATP SYNTHASE, INTEGRATION IN SIGNAL TRANSDUCTION, AND ROLE IN PATHOPHYSIOLOGY [J].

Bernardi, Paolo ;

Rasola, Andrea ;

Forte, Michael ;

Lippe, Giovanna .

PHYSIOLOGICAL REVIEWS, 2015, 95 (04) :1111-1155

[7]

Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease [J].

Casari, G ;

De Fusco, M ;

Ciarmatori, S ;

Zeviani, M ;

Mora, M ;

Fernandez, P ;

De Michele, G ;

Filla, A ;

Cocozza, S ;

Marconi, R ;

Dürr, A ;

Fontaine, B ;

Ballabio, A .

CELL, 1998, 93 (06) :973-983

[8]

A forty-kilodalton protein of the inner membrane is the mitochondrial calcium uniporter [J].

De Stefani, Diego ;

Raffaello, Anna ;

Teardo, Enrico ;

Szabo, Ildiko ;

Rizzuto, Rosario .

NATURE, 2011, 476 (7360) :336-U104

[9]

Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28 [J].

Di Bella, Daniela ;

Lazzaro, Federico ;

Brusco, Alfredo ;

Plumari, Massimo ;

Battaglia, Giorgio ;

Pastore, Annalisa ;

Finardi, Adele ;

Cagnoli, Claudia ;

Tempia, Filippo ;

Frontali, Marina ;

Veneziano, Liana ;

Sacco, Tiziana ;

Boda, Enrica ;

Brussino, Alessandro ;

Bonn, Florian ;

Castellotti, Barbara ;

Baratta, Silvia ;

Mariotti, Caterina ;

Gellera, Cinzia ;

Fracasso, Valentina ;

Magri, Stefania ;

Langer, Thomas ;

Plevani, Paolo ;

Di Donato, Stefano ;

Muzi-Falconi, Marco ;

Taroni, Franco .

NATURE GENETICS, 2010, 42 (04) :313-U66

[10]

Axonal degeneration in paraplegin-deficient mice is associated with abnormal mitochondria and impairment of axonal transport [J].

Ferreirinha, F ;

Quattrini, A ;

Pirozzi, M ;

Valsecchi, V ;

Dina, G ;

Broccoli, V ;

Auricchio, A ;

Piemonte, F ;

Tozzi, G ;

Gaeta, L ;

Casari, G ;

Ballabio, A ;

Rugarli, EI .

JOURNAL OF CLINICAL INVESTIGATION, 2004, 113 (02) :231-242

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