Quantitative connection between polyglutamine aggregation kinetics and neurodegenerative process in patients with Huntington’s disease

Gatchel JR(2005)Disease of unstable repeat expansion: mechanisms and common principles Nat Rev Genet 6 743-755

Zoghbi HY(2000)Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease Nat Rev Neurosci 1 109-115

Gusella JF(2002)Huntington’s disease age-of-onset linked to polyglutamine aggregation nucleation Proc Natl Acad Sci USA 99 11884-11889

MacDonald ME(1999)Analysis of protein aggregation kinetics Methods Enzymol 309 256-274

Chen S(2001)Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats Nature 412 143-144

Ferrone FA(2006)The two-stage pathway of ataxin-3 fibrillogenesis involves a polyglutamine-independent step J Biol Chem 281 16888-16896

Wetzel R(2009)Distinct conformations of in vitro and in vivo amyloids of huntingtin-exon1 show different cytotoxicity Proc Natl Acad Sci USA 106 9679-9684

Ferrone F(2009)Multi-domain misfolding: understanding the aggregation pathway of polyglutamine proteins Protein Eng Des sel 22 447-451

Perutz MF(1994)CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1 Nat Genet 8 221-228

Windle AH(2004)Characterization of the structure and the amyloidogenic properties of the Josephin domain of the polyglutamine-containing protein ataxin-3 J Mol Biol 344 1021-1035

Gatchel JR(2005)Disease of unstable repeat expansion: mechanisms and common principles Nat Rev Genet 6 743-755

Zoghbi HY(2000)Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease Nat Rev Neurosci 1 109-115

Gusella JF(2002)Huntington’s disease age-of-onset linked to polyglutamine aggregation nucleation Proc Natl Acad Sci USA 99 11884-11889

MacDonald ME(1999)Analysis of protein aggregation kinetics Methods Enzymol 309 256-274

Chen S(2001)Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats Nature 412 143-144

Ferrone FA(2006)The two-stage pathway of ataxin-3 fibrillogenesis involves a polyglutamine-independent step J Biol Chem 281 16888-16896

Wetzel R(2009)Distinct conformations of in vitro and in vivo amyloids of huntingtin-exon1 show different cytotoxicity Proc Natl Acad Sci USA 106 9679-9684

Ferrone F(2009)Multi-domain misfolding: understanding the aggregation pathway of polyglutamine proteins Protein Eng Des sel 22 447-451

Perutz MF(1994)CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1 Nat Genet 8 221-228

Windle AH(2004)Characterization of the structure and the amyloidogenic properties of the Josephin domain of the polyglutamine-containing protein ataxin-3 J Mol Biol 344 1021-1035