Erratum: The structure of a polyQ–anti-polyQ complex reveals binding according to a linear lattice model

Li, Pingwei; Huey-Tubman, Kathryn E.; Gao, Tiyu; Li, Xiaojun; West, Anthony P.; Bennett, M. J.; Björkman, Pamela J.

doi:10.1038/nsmb0607-568a

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(2 citation statements)

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“…This threshold varies among polyQ diseases . Consistent with these observations are the following considerations: (i) polyQ tracts longer than the toxicity threshold can form in vitro and in vivo SDS insoluble aggregates. ,,,, (ii) Specific recognition of expanded toxic polyQ tracts by an anti-polyQ monoclonal antibody has been observed, suggesting the existence of a generic conformational epitope formed only above a certain polyQ length. − …”

Section: Introductionmentioning

confidence: 59%

“…18,21,22,36,37 (ii) Specific recognition of expanded toxic polyQ tracts by an anti-polyQ monoclonal antibody has been observed, suggesting the existence of a generic conformational epitope formed only above a certain polyQ length. [38][39][40] These facts have led to the so-called "structural threshold hypothesis", stating that only above the critical length of 36-40 Qs the polyQs do undergo a structural transition from random coil to a well defined structure based on β-sheet.…”

Section: Introductionmentioning

confidence: 99%

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Structural Properties of Polyglutamine Aggregates Investigated via Molecular Dynamics Simulations

et al. 2008

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Polyglutamine (polyQ) beta-stranded aggregates constitute the hallmark of Huntington disease. The disease is fully penetrant when Q residues are more than 36-40 ("disease threshold"). Here, based on a molecular dynamics study on polyQ helical structures of different shapes and oligomeric states, we suggest that the stability of the aggregates increases with the number of monomers, while it is rather insensitive to the number of Qs in each monomer. However, the stability of the single monomer does depend on the number of side-chain intramolecular H-bonds, and therefore on the number of Qs. If such number is lower than that of the disease threshold, the beta-stranded monomers are unstable and hence may aggregate with lower probability, consistently with experimental findings. Our results provide a possible interpretation of the apparent polyQ length dependent-toxicity, and they do not support the so-called "structural threshold hypothesis", which supposes a transition from random coil to a beta-sheet structure only above the disease threshold.

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