Ubiquitin-interacting motifs of ataxin-3 regulate its polyglutamine toxicity through Hsc70-4-dependent aggregation.
D. melanogaster
aggregation
ataxia
cell biology
deubiquitinase
machado-joseph disease
neurodegeneration
neuroscience
proteostasis
Journal
eLife
ISSN: 2050-084X
Titre abrégé: Elife
Pays: England
ID NLM: 101579614
Informations de publication
Date de publication:
21 09 2020
21 09 2020
Historique:
received:
06
07
2020
accepted:
30
08
2020
entrez:
21
9
2020
pubmed:
22
9
2020
medline:
2
3
2021
Statut:
epublish
Résumé
Spinocerebellar ataxia type 3 (SCA3) belongs to the family of polyglutamine neurodegenerations. Each disorder stems from the abnormal lengthening of a glutamine repeat in a different protein. Although caused by a similar mutation, polyglutamine disorders are distinct, implicating non-polyglutamine regions of disease proteins as regulators of pathogenesis. SCA3 is caused by polyglutamine expansion in ataxin-3. To determine the role of ataxin-3's non-polyglutamine domains in disease, we utilized a new, allelic series of
Identifiants
pubmed: 32955441
doi: 10.7554/eLife.60742
pii: 60742
pmc: PMC7505662
doi:
pii:
Substances chimiques
Drosophila Proteins
0
HSC70 Heat-Shock Proteins
0
Hsc70-4 protein, Drosophila
0
Peptides
0
Ubiquitin
0
polyglutamine
26700-71-0
Ataxin-3
EC 3.4.19.12
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : NINDS NIH HHS
ID : R01 NS086778
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS086773
Pays : United States
Informations de copyright
© 2020, Johnson et al.
Déclaration de conflit d'intérêts
SJ, BR, KL, WT, ST No competing interests declared
Références
Aging (Albany NY). 2015 Aug;7(8):535-52
pubmed: 26298685
J Biol Chem. 2010 Dec 10;285(50):39303-13
pubmed: 20943656
J Neurosci. 2009 Sep 30;29(39):12079-88
pubmed: 19793966
Subcell Biochem. 2015;78:243-73
pubmed: 25487025
Methods Cell Biol. 1994;44:635-54
pubmed: 7707973
Hum Mol Genet. 2002 Nov 1;11(23):2895-904
pubmed: 12393801
Annu Rev Pathol. 2019 Jan 24;14:1-27
pubmed: 30089230
Hum Mol Genet. 2015 Aug 1;24(15):4385-96
pubmed: 25954029
Genetics. 2004 Apr;166(4):1775-82
pubmed: 15126397
Nucleic Acids Res. 2003 Oct 1;31(19):5469-82
pubmed: 14500809
Nat Genet. 1999 Dec;23(4):425-8
pubmed: 10581028
FASEB J. 2013 Apr;27(4):1450-9
pubmed: 23271055
Nat Biotechnol. 2010 Mar;28(3):256-63
pubmed: 20190739
PLoS One. 2009 Jun 08;4(6):e5834
pubmed: 19503814
Biopolymers. 2009 Dec;91(12):1203-14
pubmed: 19382171
Cell Rep. 2014 May 8;7(3):774-84
pubmed: 24746732
Hum Mol Genet. 2011 Dec 15;20(24):4810-21
pubmed: 21933837
J Biol Chem. 2005 Sep 9;280(36):32026-34
pubmed: 16040601
Cell. 1998 Jun 12;93(6):939-49
pubmed: 9635424
Nat Rev Dis Primers. 2019 Apr 11;5(1):24
pubmed: 30975995
Autophagy. 2013 Dec;9(12):1937-54
pubmed: 24121476
Hum Mol Genet. 2015 Jul 15;24(14):3971-81
pubmed: 25882706
Mol Cell. 2017 Jan 5;65(1):52-65
pubmed: 27916661
Nature. 2008 Jun 19;453(7198):1107-11
pubmed: 18449188
J Biol Chem. 2006 Jun 23;281(25):16888-96
pubmed: 16624810
EMBO J. 2006 Apr 5;25(7):1547-58
pubmed: 16525503
PLoS One. 2014 Apr 14;9(4):e94322
pubmed: 24732912
Proc Natl Acad Sci U S A. 2011 Jan 4;108(1):260-5
pubmed: 21173221
Hum Mol Genet. 2017 Apr 15;26(8):1419-1431
pubmed: 28158474
PLoS One. 2012;7(11):e47452
pubmed: 23139745
J Biol Chem. 2007 Oct 5;282(40):29348-58
pubmed: 17693639
Neuron. 1997 Aug;19(2):333-44
pubmed: 9292723
Lancet Neurol. 2016 Jun;15(7):748-759
pubmed: 27106072
Hum Mol Genet. 2000 Nov 22;9(19):2811-20
pubmed: 11092757
Neurobiol Dis. 2018 Aug;116:78-92
pubmed: 29704548
J Biol Chem. 2013 Nov 29;288(48):34460-9
pubmed: 24106274
Hum Mol Genet. 2002 Aug 15;11(17):1967-76
pubmed: 12165558
Cell Rep. 2016 Sep 27;17(1):125-136
pubmed: 27681426
Mol Cell. 2020 Apr 16;78(2):346-358.e9
pubmed: 32268123
Cells. 2019 Oct 16;8(10):
pubmed: 31623164
Neurobiol Dis. 2019 Dec;132:104535
pubmed: 31310802
J Mol Biol. 2004 Dec 3;344(4):1021-35
pubmed: 15544810
Proc Natl Acad Sci U S A. 2005 Jul 26;102(30):10493-8
pubmed: 16020535
Nat Rev Mol Cell Biol. 2009 Oct;10(10):659-71
pubmed: 19773779
Genetics. 2010 Apr;184(4):1165-79
pubmed: 20100940
Annu Rev Biochem. 2017 Jun 20;86:129-157
pubmed: 28375744
J Neurosci. 2007 Jul 11;27(28):7418-28
pubmed: 17626202
Neuron. 2015 Nov 18;88(4):667-77
pubmed: 26590344
J Biol Chem. 2008 Sep 26;283(39):26436-43
pubmed: 18599482
Oncogene. 2004 Mar 15;23(11):2057-70
pubmed: 15021893
PLoS Genet. 2007 Oct;3(10):1950-64
pubmed: 17953484
Cell Mol Life Sci. 2020 Mar;77(6):977-996
pubmed: 31552448
Essays Biochem. 2005;41:49-67
pubmed: 16250897
J Biol Chem. 1999 Jan 8;274(2):781-6
pubmed: 9873016
Nat Commun. 2014 Aug 21;5:4638
pubmed: 25144244
Mol Cell. 2005 Apr 1;18(1):37-48
pubmed: 15808507
Proc Natl Acad Sci U S A. 2005 Jul 19;102(29):10261-6
pubmed: 16009936
EMBO J. 2018 Jan 17;37(2):282-299
pubmed: 29212816
Mol Neurodegener. 2013 Oct 27;8:42
pubmed: 24160175
PLoS One. 2010 Oct 27;5(10):e13695
pubmed: 21060878
Neurobiol Dis. 2015 Oct;82:12-21
pubmed: 26007638
Development. 1993 Jun;118(2):401-15
pubmed: 8223268
Genesis. 2002 Sep-Oct;34(1-2):127-31
pubmed: 12324966
Genetics. 2008 Jan;178(1):215-34
pubmed: 18202369
J Med Chem. 2010 Jun 24;53(12):4585-602
pubmed: 20334364
Cold Spring Harb Perspect Biol. 2018 Dec 3;10(12):
pubmed: 29891563
J Biol Chem. 2015 Jan 30;290(5):2560-76
pubmed: 25505179
Mol Biol Cell. 2011 Feb 1;22(3):301-14
pubmed: 21148293
Biomolecules. 2020 Aug 04;10(8):
pubmed: 32759676
PLoS One. 2010 Aug 26;5(8):e12430
pubmed: 20865150
Front Mol Neurosci. 2017 Mar 22;10:78
pubmed: 28381987
Cell. 1998 Oct 2;95(1):41-53
pubmed: 9778246
Adv Exp Med Biol. 2020;1233:237-260
pubmed: 32274760
Hum Mol Genet. 2012 May 15;21(10):2211-8
pubmed: 22337953
Adv Exp Med Biol. 2018;1049:135-145
pubmed: 29427101
EMBO J. 2009 Feb 18;28(4):372-82
pubmed: 19153604
Cell Struct Funct. 2012;37(2):177-87
pubmed: 23018488
Proc Natl Acad Sci U S A. 2001 Oct 23;98(22):12596-601
pubmed: 11675495
Adv Exp Med Biol. 2020;1243:3-20
pubmed: 32297208
J Biol Chem. 2020 May 22;295(21):7301-7316
pubmed: 32284329
Brain Res. 2016 Sep 15;1647:30-42
pubmed: 27060770
Sci Rep. 2018 May 31;8(1):8513
pubmed: 29855490
Nat Genet. 1994 Nov;8(3):221-8
pubmed: 7874163
Int J Mol Sci. 2015 Aug 11;16(8):18741-51
pubmed: 26270660
Biol Open. 2016 Dec 15;5(12):1770-1775
pubmed: 27979829
J Biol Chem. 2005 Feb 4;280(5):3898-910
pubmed: 15533937
Cell Struct Funct. 2010;35(2):107-16
pubmed: 21150129
Biophys J. 2011 Apr 20;100(8):2033-42
pubmed: 21504740
Front Aging Neurosci. 2019 Mar 11;11:49
pubmed: 30914945
Brain Pathol. 2016 Nov;26(6):779-786
pubmed: 27529325
Prog Neurobiol. 2012 May;97(2):239-57
pubmed: 22133674