Sort by
Items per page

Send to

Choose Destination

Links from PubMed

Items: 1 to 20 of 29


Multifunctional Mitochondrial AAA Proteases.

Glynn SE.

Front Mol Biosci. 2017 May 22;4:34. doi: 10.3389/fmolb.2017.00034. eCollection 2017. Review.


An immunoaffinity-based method for isolating ultrapure adult astrocytes based on ATP1B2 targeting by the ACSA-2 antibody.

Batiuk MY, de Vin F, Duqué SI, Li C, Saito T, Saido T, Fiers M, Belgard TG, Holt MG.

J Biol Chem. 2017 May 26;292(21):8874-8891. doi: 10.1074/jbc.M116.765313. Epub 2017 Apr 3.


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 2;12(12):e1006463. doi: 10.1371/journal.pgen.1006463. eCollection 2016 Dec.


Caveolin-1 controls mitochondrial function through regulation of m-AAA mitochondrial protease.

Volonte D, Liu Z, Shiva S, Galbiati F.

Aging (Albany NY). 2016 Oct 4;8(10):2355-2369. doi: 10.18632/aging.101051.


Cerebellar Ataxia and Coenzyme Q Deficiency through Loss of Unorthodox Kinase Activity.

Stefely JA, Licitra F, Laredj L, Reidenbach AG, Kemmerer ZA, Grangeray A, Jaeg-Ehret T, Minogue CE, Ulbrich A, Hutchins PD, Wilkerson EM, Ruan Z, Aydin D, Hebert AS, Guo X, Freiberger EC, Reutenauer L, Jochem A, Chergova M, Johnson IE, Lohman DC, Rush MJP, Kwiecien NW, Singh PK, Schlagowski AI, Floyd BJ, Forsman U, Sindelar PJ, Westphall MS, Pierrel F, Zoll J, Dal Peraro M, Kannan N, Bingman CA, Coon JJ, Isope P, Puccio H, Pagliarini DJ.

Mol Cell. 2016 Aug 18;63(4):608-620. doi: 10.1016/j.molcel.2016.06.030. Epub 2016 Aug 4.


Mitochondrial Quality Control Proteases in Neuronal Welfare.

Levytskyy RM, Germany EM, Khalimonchuk O.

J Neuroimmune Pharmacol. 2016 Dec;11(4):629-644. Epub 2016 May 2. Review.


Cerebellar ataxias: β-III spectrin's interactions suggest common pathogenic pathways.

Perkins E, Suminaite D, Jackson M.

J Physiol. 2016 Aug 15;594(16):4661-76. doi: 10.1113/JP271195. Epub 2016 Apr 24. Review.


Mitochondrial ribosome assembly in health and disease.

De Silva D, Tu YT, Amunts A, Fontanesi F, Barrientos A.

Cell Cycle. 2015;14(14):2226-50. doi: 10.1080/15384101.2015.1053672. Epub 2015 Jun 1. Review.


Therapeutic prospects for spinocerebellar ataxia type 2 and 3.

Bezprozvanny I, Klockgether T.

Drugs Future. 2009 Dec;34(12). doi: 10.1358/dof.2009.034.12.1443434.


Mitochondrial Diseases Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function.

Iommarini L, Peralta S, Torraco A, Diaz F.

Mitochondrion. 2015 May;22:96-118. doi: 10.1016/j.mito.2015.01.008. Epub 2015 Jan 29. Review.


Damaged mitochondria in Fanconi anemia - an isolated event or a general phenomenon?

Pagano G, Shyamsunder P, Verma RS, Lyakhovich A.

Oncoscience. 2014 Apr 21;1(4):287-95. eCollection 2014.


Purkinje neuron Ca2+ influx reduction rescues ataxia in SCA28 model.

Maltecca F, Baseggio E, Consolato F, Mazza D, Podini P, Young SM Jr, Drago I, Bahr BA, Puliti A, Codazzi F, Quattrini A, Casari G.

J Clin Invest. 2015 Jan;125(1):263-74. doi: 10.1172/JCI74770. Epub 2014 Dec 8.


Mitochondrial dysfunction-associated OPA1 cleavage contributes to muscle degeneration: preventative effect of hydroxytyrosol acetate.

Wang X, Li H, Zheng A, Yang L, Liu J, Chen C, Tang Y, Zou X, Li Y, Long J, Liu J, Zhang Y, Feng Z.

Cell Death Dis. 2014 Nov 13;5:e1521. doi: 10.1038/cddis.2014.473.


Lgr4 protein deficiency induces ataxia-like phenotype in mice and impairs long term depression at cerebellar parallel fiber-Purkinje cell synapses.

Guan X, Duan Y, Zeng Q, Pan H, Qian Y, Li D, Cao X, Liu M.

J Biol Chem. 2014 Sep 19;289(38):26492-504. doi: 10.1074/jbc.M114.564138. Epub 2014 Jul 25.


Loss of the m-AAA protease subunit AFG₃L₂ causes mitochondrial transport defects and tau hyperphosphorylation.

Kondadi AK, Wang S, Montagner S, Kladt N, Korwitz A, Martinelli P, Herholz D, Baker MJ, Schauss AC, Langer T, Rugarli EI.

EMBO J. 2014 May 2;33(9):1011-26. doi: 10.1002/embj.201387009. Epub 2014 Mar 28.


The histone deacetylase HDAC3 is essential for Purkinje cell function, potentially complicating the use of HDAC inhibitors in SCA1.

Venkatraman A, Hu YS, Didonna A, Cvetanovic M, Krbanjevic A, Bilesimo P, Opal P.

Hum Mol Genet. 2014 Jul 15;23(14):3733-45. doi: 10.1093/hmg/ddu081. Epub 2014 Mar 4.


Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias.

Matilla-Dueñas A, Ashizawa T, Brice A, Magri S, McFarland KN, Pandolfo M, Pulst SM, Riess O, Rubinsztein DC, Schmidt J, Schmidt T, Scoles DR, Stevanin G, Taroni F, Underwood BR, Sánchez I.

Cerebellum. 2014 Apr;13(2):269-302. doi: 10.1007/s12311-013-0539-y.


Hereditary spastic paraplegia: clinico-pathologic features and emerging molecular mechanisms.

Fink JK.

Acta Neuropathol. 2013 Sep;126(3):307-28. doi: 10.1007/s00401-013-1115-8. Epub 2013 Jul 30. Review.


Genome-wide expression profiling and functional characterization of SCA28 lymphoblastoid cell lines reveal impairment in cell growth and activation of apoptotic pathways.

Mancini C, Roncaglia P, Brussino A, Stevanin G, Lo Buono N, Krmac H, Maltecca F, Gazzano E, Bartoletti Stella A, Calvaruso MA, Iommarini L, Cagnoli C, Forlani S, Le Ber I, Durr A, Brice A, Ghigo D, Casari G, Porcelli AM, Funaro A, Gasparre G, Gustincich S, Brusco A.

BMC Med Genomics. 2013 Jun 18;6:22. doi: 10.1186/1755-8794-6-22.


Selective positive modulator of calcium-activated potassium channels exerts beneficial effects in a mouse model of spinocerebellar ataxia type 2.

Kasumu AW, Hougaard C, Rode F, Jacobsen TA, Sabatier JM, Eriksen BL, Strøbæk D, Liang X, Egorova P, Vorontsova D, Christophersen P, Rønn LC, Bezprozvanny I.

Chem Biol. 2012 Oct 26;19(10):1340-53. doi: 10.1016/j.chembiol.2012.07.013.

Supplemental Content

Support Center