Results 41 to 50 of about 9,929 (238)

NEDD8: A new ataxin-3 interactor

Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, 2007
Machado-Joseph disease (MJD/SCA3) is an autosomal dominant neurodegenerative disease caused by the expansion of a CAG tract in the coding portion of the ATXN3 gene. The presence of ubiquitin-positive aggregates of the defective protein in affected neurons is characteristic of this and most of the polyglutamine disorders.
Ferro, Anabela, Carvalho, Ana Luísa, Teixeira-Castro, Andreia, Almeida, Carla, Tomé, Ricardo J., Cortes, Luísa, Rodrigues, Ana-João, Logarinho, Elsa, Sequeiros, Jorge, Macedo-Ribeiro, Sandra, Maciel, Patrícia   +10 more
openaire   +5 more sources

Trehalose alleviates the phenotype of Machado–Joseph disease mouse models [PDF]

, 2020
Machado-Joseph disease (MJD), also known as spinocerebellar ataxia type 3, is the most common of the dominantly inherited ataxias worldwide and is characterized by mutant ataxin-3 aggregation and neuronal degeneration.
Cavadas, Cláudia, Cunha-Santos, Janete, Gaspar, Laetitia S., Greif, Hagar, Nobre, Rui Jorge, Nóbrega, Clévio, Paixão, Susana, Pereira de Almeida, Luís, Santana, Magda M., Silva, Teresa Pereira, Trevino-Garcia, Allyson   +10 more
core   +1 more source

The cholesterol 24-hydroxylase activates autophagy and decreases mutant huntingtin build-up in a neuroblastoma culture model of Huntington’s disease [PDF]

, 2020
Objective Compromised brain cholesterol turnover and altered regulation of brain cholesterol metabolism have been allied with some neurodegenerative diseases, including Huntington’s disease (HD).
Alves, Sandro, Betuing, Sandrine, Caboche, Jocelyne, Carmo-Silva, Sara, Cartier, Nathalie, Conceição, André, Costa, Rafael G, Koppenol, Rebekah, Marcelo, Adriana, Matos, Carlos A, Nunes, Ricardo, Nóbrega, Clévio, Sequeira, Raquel L.   +12 more
core   +1 more source

Autophagy mediates SUMO-induced degradation of a polyglutamine protein ataxin-3. [PDF]

Anim Cells Syst (Seoul), 2017
Hwang SP, Lee DH.
europepmc   +3 more sources

Calpain inhibition reduces ataxin-3 cleavage alleviating neuropathology and motor impairments in mouse models of Machado-Joseph disease [PDF]

, 2014
Machado–Joseph Disease (MJD) is the most prevalent autosomal dominantly inherited cerebellar ataxia. It is caused by an expanded CAG repeat in the ATXN3 gene, which translates into a polyglutamine tract within the ataxin-3 protein. Present treatments are
Almeida, Luís Pereira de, Duarte, Carlos Bandeira, Gonçalves, Nélio, Nobre, Rui Jorge, Simões, Ana Teresa   +4 more
core   +1 more source

Towards a structural understanding of the fibrillization pathway in Machado-Joseph’s disease: trapping early oligomers of non-expanded ataxin-3 [PDF]

, 2005
Machado-Joseph’s disease is caused by a CAG trinucleotide repeat expansion that is translated into an abnormally long polyglutamine tract in the protein ataxin-3.
Almeida, Carla, Clarke, David T., Cortes, Luísa, Costa, Maria do Carmo, Damas, Ana Margarida, Gales, Luís, Maciel, P., Melo, Carlos V., Ribeiro, Sandra Macedo   +8 more
core   +1 more source

Examination of ataxin-3 (atx-3) aggregation by structural mass spectrometry techniques: A rationale for expedited aggregation upon polyglutamine (polyQ) expansion [PDF]

, 2015
Expansion of polyglutamine stretches leads to the formation of polyglutamine-containing neuronal aggregates and neuronal death in nine diseases for which there currently are no treatments or cures.
Alison E. Ashcroft, Almeida, Ashcroft, Bernstein, Beveridge, Beveridge, Bevivino, Bleiholder, Bruno Almeida, Bush, Charlotte A. Scarff, Chow, Chow, Costa, Cummings, de Chiara, Duennwald, Ellisdon, Ellisdon, England, Fujigasaki, Gales, Gerhard, Goto, Hilton, Ignatova, Invernizzi, Ivanova, Joana Fraga, Kettner, Lajoie, Masino, Masino, Masino, Matos, Menon, Nagai, Natalello, Nicastro, Nicastro, Orr, Paulson, Rockabrand, Ruotolo, Ruotolo, Sandra Macedo-Ribeiro, Sanfelice, Saunders, Saunders, Scarff, Sheena E. Radford, Smith, Spitzer, Testa, Thakur, Zoghbi   +55 more
core   +1 more source

Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties. [PDF]

PLoS ONE, 2010
Protein context clearly influences neurotoxicity in polyglutamine diseases, but the contribution of alternative splicing to this phenomenon has rarely been investigated.
Ginny Marie Harris, Katerina Dodelzon, Lijie Gong, Pedro Gonzalez-Alegre, Henry L Paulson   +4 more
doaj   +1 more source

Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3 [PDF]

, 2008
Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD), is an autosomal-dominant neurodegenerative disorder caused by a polyglutamine expansion in ataxin-3 (SCA3, MJD1) protein.
Bezprozvanny, I, Chen, X, Hammer, R, Nelson, O, Nukina, N, Pook, M, Tang, TS, Tu, H   +7 more
core   +1 more source

Caffeine and adenosine A 2A receptor inactivation decrease striatal neuropathology in a lentiviral-based model of Machado–Joseph disease [PDF]

, 2013
Objective: Machado–Joseph disease (MJD) is a neurodegenerative disorder associated with an abnormal CAG expansion, which translates into an expanded polyglutamine tract within ataxin-3.
Almeida, Luís Pereira de, Cunha, Rodrigo A., Gonçalves, Nélio, Simões, Ana T.   +3 more
core   +1 more source