The rate of polyQ-mediated aggregation is dramatically affected by the number and location of surrounding domains.

[1]  Ashley M. Buckle,et al.  PolyQ: a database describing the sequence and domain context of polyglutamine repeats in proteins , 2010, Nucleic Acids Res..

[2]  Martin J. Scanlon,et al.  Small heat-shock proteins interact with a flanking domain to suppress polyglutamine aggregation , 2010, Proceedings of the National Academy of Sciences.

[3]  Regina M Murphy,et al.  Examining polyglutamine peptide length: a connection between collapsed conformations and increased aggregation. , 2009, Journal of molecular biology.

[4]  Judith Frydman,et al.  The Chaperonin TRIC Blocks a Huntingtin Sequence Element that promotes the Conformational Switch to Aggregation , 2009, Nature Structural &Molecular Biology.

[5]  Zbyszek Otwinowski,et al.  Secondary structure of Huntingtin amino-terminal region. , 2009, Structure.

[6]  Dalaver H. Anjum,et al.  Polyglutamine disruption of the huntingtin exon1 N-terminus triggers a complex aggregation mechanism , 2009, Nature Structural &Molecular Biology.

[7]  Amy L. Robertson,et al.  The structural impact of a polyglutamine tract is location-dependent. , 2008, Biophysical journal.

[8]  E. Kremmer,et al.  Dynamic interactions of Sup35p and PrP prion protein domains modulate aggregate nucleation and seeding , 2008, Prion.

[9]  Sven Rahmann,et al.  Large scale clustering of protein sequences with FORCE -A layout based heuristic for weighted cluster editing , 2007, BMC Bioinformatics.

[10]  Mariko Y Momoi,et al.  Intracellular distribution of a speech/language disorder associated FOXP2 mutant. , 2007, Biochemical and biophysical research communications.

[11]  Chien-Yu Chen,et al.  Exploiting homogeneity in protein sequence clusters for construction of protein family hierarchies , 2006, Pattern Recognit..

[12]  Stephen P Bottomley,et al.  The Two-stage Pathway of Ataxin-3 Fibrillogenesis Involves a Polyglutamine-independent Step* , 2006, Journal of Biological Chemistry.

[13]  Wei Li,et al.  Expression and Characterization of Full-length Human Huntingtin, an Elongated HEAT Repeat Protein* , 2006, Journal of Biological Chemistry.

[14]  Frederic Rousseau,et al.  How evolutionary pressure against protein aggregation shaped chaperone specificity. , 2006, Journal of molecular biology.

[15]  L. Serrano,et al.  Protein aggregation and amyloidosis: confusion of the kinds? , 2006, Current opinion in structural biology.

[16]  Ronald Wetzel,et al.  Oligoproline effects on polyglutamine conformation and aggregation. , 2006, Journal of molecular biology.

[17]  S. Teichmann,et al.  The importance of sequence diversity in the aggregation and evolution of proteins , 2005, Nature.

[18]  L. Holm,et al.  The Pfam protein families database , 2005, Nucleic Acids Res..

[19]  J. Whisstock,et al.  Functional insights from the distribution and role of homopeptide repeat-containing proteins. , 2005, Genome research.

[20]  S. Brahmachari,et al.  Identification of a novel 45 repeat unstable allele associated with a disease phenotype at the MJD1/SCA3 locus , 2005, American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics.

[21]  A. Fersht,et al.  Testing protein-folding simulations by experiment: B domain of protein A. , 2004, Proceedings of the National Academy of Sciences of the United States of America.

[22]  C. Dobson,et al.  Protein aggregation and aggregate toxicity: new insights into protein folding, misfolding diseases and biological evolution , 2003, Journal of Molecular Medicine.

[23]  A. Horwich Protein aggregation in disease: a role for folding intermediates forming specific multimeric interactions. , 2002, The Journal of clinical investigation.

[24]  Ronald Wetzel,et al.  Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation , 2002, Proceedings of the National Academy of Sciences of the United States of America.

[25]  Ronald Wetzel,et al.  Amyloid-like features of polyglutamine aggregates and their assembly kinetics. , 2002, Biochemistry.

[26]  S. Dongen Graph clustering by flow simulation , 2000 .

[27]  D. Eisenberg,et al.  A census of protein repeats. , 1999, Journal of molecular biology.

[28]  R. Glockshuber,et al.  Influence of amino acid substitutions related to inherited human prion diseases on the thermodynamic stability of the cellular prion protein. , 1999, Biochemistry.

[29]  A. Hackam,et al.  Kennedy's Disease , 1999, Journal of neurochemistry.

[30]  W. Surewicz,et al.  Familial Mutations and the Thermodynamic Stability of the Recombinant Human Prion Protein* , 1998, The Journal of Biological Chemistry.

[31]  T. Klockgether,et al.  An Isoform of Ataxin‐3 Accumulates in the Nucleus of Neuronal Cells in Affected Brain Regions of SCA3 Patients , 1998, Brain pathology.

[32]  Dale E. Bredesen,et al.  Caspase Cleavage of Gene Products Associated with Triplet Expansion Disorders Generates Truncated Fragments Containing the Polyglutamine Tract* , 1998, The Journal of Biological Chemistry.

[33]  R. Albin,et al.  Ectopically Expressed CAG Repeats Cause Intranuclear Inclusions and a Progressive Late Onset Neurological Phenotype in the Mouse , 1997, Cell.

[34]  S. W. Davies,et al.  Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. , 1997, Science.

[35]  Masahiko Watanabe,et al.  Machado–Joseph disease gene products carrying different carboxyl termini , 1997, Neuroscience Research.

[36]  M. McInnis,et al.  cDNAs with long CAG trinucleotide repeats from human brain , 1997, Human Genetics.

[37]  S. Bottomley,et al.  The stability and unfolding of an IgG binding protein based upon the B domain of protein A from Staphylococcus aureus probed by tryptophan substitution and fluorescence spectroscopy. , 1994, Protein engineering.

[38]  J T Finch,et al.  Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases. , 1994, Proceedings of the National Academy of Sciences of the United States of America.

[39]  M. Hurle,et al.  A role for destabilizing amino acid replacements in light-chain amyloidosis. , 1994, Proceedings of the National Academy of Sciences of the United States of America.

[40]  E. Myers,et al.  Basic local alignment search tool. , 1990, Journal of molecular biology.