265 related articles for article (PubMed ID: 30989755)
1. Astrocyte-specific deletion of the mitochondrial m-AAA protease reveals glial contribution to neurodegeneration.
Murru S; Hess S; Barth E; Almajan ER; Schatton D; Hermans S; Brodesser S; Langer T; Kloppenburg P; Rugarli EI
Glia; 2019 Aug; 67(8):1526-1541. PubMed ID: 30989755
[TBL] [Abstract][Full Text] [Related]
2. m-AAA proteases, mitochondrial calcium homeostasis and neurodegeneration.
Patron M; Sprenger HG; Langer T
Cell Res; 2018 Mar; 28(3):296-306. PubMed ID: 29451229
[TBL] [Abstract][Full Text] [Related]
3. Concurrent AFG3L2 and SPG7 mutations associated with syndromic parkinsonism and optic atrophy with aberrant OPA1 processing and mitochondrial network fragmentation.
Magri S; Fracasso V; Plumari M; Alfei E; Ghezzi D; Gellera C; Rusmini P; Poletti A; Di Bella D; Elia AE; Pantaleoni C; Taroni F
Hum Mutat; 2018 Dec; 39(12):2060-2071. PubMed ID: 30252181
[TBL] [Abstract][Full Text] [Related]
4. The Mitochondrial m-AAA Protease Prevents Demyelination and Hair Greying.
Wang S; Jacquemyn J; Murru S; Martinelli P; Barth E; Langer T; Niessen CM; Rugarli EI
PLoS Genet; 2016 Dec; 12(12):e1006463. PubMed ID: 27911893
[TBL] [Abstract][Full Text] [Related]
5. AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival.
Almajan ER; Richter R; Paeger L; Martinelli P; Barth E; Decker T; Larsson NG; Kloppenburg P; Langer T; Rugarli EI
J Clin Invest; 2012 Nov; 122(11):4048-58. PubMed ID: 23041622
[TBL] [Abstract][Full Text] [Related]
6. The m-AAA Protease Associated with Neurodegeneration Limits MCU Activity in Mitochondria.
König T; Tröder SE; Bakka K; Korwitz A; Richter-Dennerlein R; Lampe PA; Patron M; Mühlmeister M; Guerrero-Castillo S; Brandt U; Decker T; Lauria I; Paggio A; Rizzuto R; Rugarli EI; De Stefani D; Langer T
Mol Cell; 2016 Oct; 64(1):148-162. PubMed ID: 27642048
[TBL] [Abstract][Full Text] [Related]
7. Mice harbouring a SCA28 patient mutation in AFG3L2 develop late-onset ataxia associated with enhanced mitochondrial proteotoxicity.
Mancini C; Hoxha E; Iommarini L; Brussino A; Richter U; Montarolo F; Cagnoli C; Parolisi R; Gondor Morosini DI; Nicolò V; Maltecca F; Muratori L; Ronchi G; Geuna S; Arnaboldi F; Donetti E; Giorgio E; Cavalieri S; Di Gregorio E; Pozzi E; Ferrero M; Riberi E; Casari G; Altruda F; Turco E; Gasparre G; Battersby BJ; Porcelli AM; Ferrero E; Brusco A; Tempia F
Neurobiol Dis; 2019 Apr; 124():14-28. PubMed ID: 30389403
[TBL] [Abstract][Full Text] [Related]
8. Whole-exome sequencing identifies homozygous AFG3L2 mutations in a spastic ataxia-neuropathy syndrome linked to mitochondrial m-AAA proteases.
Pierson TM; Adams D; Bonn F; Martinelli P; Cherukuri PF; Teer JK; Hansen NF; Cruz P; Mullikin For The Nisc Comparative Sequencing Program JC; Blakesley RW; Golas G; Kwan J; Sandler A; Fuentes Fajardo K; Markello T; Tifft C; Blackstone C; Rugarli EI; Langer T; Gahl WA; Toro C
PLoS Genet; 2011 Oct; 7(10):e1002325. PubMed ID: 22022284
[TBL] [Abstract][Full Text] [Related]
9. Mouse brain expression patterns of Spg7, Afg3l1, and Afg3l2 transcripts, encoding for the mitochondrial m-AAA protease.
Sacco T; Boda E; Hoxha E; Pizzo R; Cagnoli C; Brusco A; Tempia F
BMC Neurosci; 2010 Apr; 11():55. PubMed ID: 20426821
[TBL] [Abstract][Full Text] [Related]
10. Pathogenic variants in the AFG3L2 proteolytic domain cause SCA28 through haploinsufficiency and proteostatic stress-driven OMA1 activation.
Tulli S; Del Bondio A; Baderna V; Mazza D; Codazzi F; Pierson TM; Ambrosi A; Nolte D; Goizet C; Toro C; Baets J; Deconinck T; DeJonghe P; Mandich P; Casari G; Maltecca F
J Med Genet; 2019 Aug; 56(8):499-511. PubMed ID: 30910913
[TBL] [Abstract][Full Text] [Related]
11. Haploinsufficiency of AFG3L2, the gene responsible for spinocerebellar ataxia type 28, causes mitochondria-mediated Purkinje cell dark degeneration.
Maltecca F; Magnoni R; Cerri F; Cox GA; Quattrini A; Casari G
J Neurosci; 2009 Jul; 29(29):9244-54. PubMed ID: 19625515
[TBL] [Abstract][Full Text] [Related]
12. Loss of Mitochondrial AAA Proteases AFG3L2 and YME1L Impairs Mitochondrial Structure and Respiratory Chain Biogenesis.
Cesnekova J; Rodinova M; Hansikova H; Zeman J; Stiburek L
Int J Mol Sci; 2018 Dec; 19(12):. PubMed ID: 30544562
[TBL] [Abstract][Full Text] [Related]
13. Purkinje neuron Ca2+ influx reduction rescues ataxia in SCA28 model.
Maltecca F; Baseggio E; Consolato F; Mazza D; Podini P; Young SM; Drago I; Bahr BA; Puliti A; Codazzi F; Quattrini A; Casari G
J Clin Invest; 2015 Jan; 125(1):263-74. PubMed ID: 25485680
[TBL] [Abstract][Full Text] [Related]
14. Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28.
Di Bella D; Lazzaro F; Brusco A; Plumari M; Battaglia G; Pastore A; Finardi A; Cagnoli C; Tempia F; Frontali M; Veneziano L; Sacco T; Boda E; Brussino A; Bonn F; Castellotti B; Baratta S; Mariotti C; Gellera C; Fracasso V; Magri S; Langer T; Plevani P; Di Donato S; Muzi-Falconi M; Taroni F
Nat Genet; 2010 Apr; 42(4):313-21. PubMed ID: 20208537
[TBL] [Abstract][Full Text] [Related]
15. Autocatalytic processing of m-AAA protease subunits in mitochondria.
Koppen M; Bonn F; Ehses S; Langer T
Mol Biol Cell; 2009 Oct; 20(19):4216-24. PubMed ID: 19656850
[TBL] [Abstract][Full Text] [Related]
16. Sustained OMA1-mediated integrated stress response is beneficial for spastic ataxia type 5.
Franchino CA; Brughera M; Baderna V; De Ritis D; Rocco A; Seneca S; Regal L; Podini P; D'Antonio M; Toro C; Quattrini A; Scalais E; Maltecca F
Brain; 2024 Mar; 147(3):1043-1056. PubMed ID: 37804316
[TBL] [Abstract][Full Text] [Related]
17. Transient IKK2 activation in astrocytes initiates selective non-cell-autonomous neurodegeneration.
Lattke M; Reichel SN; Magnutzki A; Abaei A; Rasche V; Walther P; Calado DP; Ferger B; Wirth T; Baumann B
Mol Neurodegener; 2017 Feb; 12(1):16. PubMed ID: 28193238
[TBL] [Abstract][Full Text] [Related]
18. Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia.
Koppen M; Metodiev MD; Casari G; Rugarli EI; Langer T
Mol Cell Biol; 2007 Jan; 27(2):758-67. PubMed ID: 17101804
[TBL] [Abstract][Full Text] [Related]
19. A novel AFG3L2 mutation close to AAA domain leads to aberrant OMA1 and OPA1 processing in a family with optic atrophy.
Baderna V; Schultz J; Kearns LS; Fahey M; Thompson BA; Ruddle JB; Huq A; Maltecca F
Acta Neuropathol Commun; 2020 Jun; 8(1):93. PubMed ID: 32600459
[TBL] [Abstract][Full Text] [Related]
20. Respiratory dysfunction by AFG3L2 deficiency causes decreased mitochondrial calcium uptake via organellar network fragmentation.
Maltecca F; De Stefani D; Cassina L; Consolato F; Wasilewski M; Scorrano L; Rizzuto R; Casari G
Hum Mol Genet; 2012 Sep; 21(17):3858-70. PubMed ID: 22678058
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]