HEAT repeats in the Huntington's disease protein

  title={HEAT repeats in the Huntington's disease protein},
  author={Miguel Andrade and Peer Bork},
  journal={Nature Genetics},
CRMP1 protein complexes modulate polyQ-mediated Htt aggregation and toxicity in neurons
The focus of this thesis was to investigate the role of the CRMP1 protein in HD pathogenesis using in vitro and in vivo model systems and found new findings on the function ofCRMP1 and its role in the pathogenesis of HD.
The PCI domains are “winged” HEAT domains
Evidence is presented that the PCI domains as a group have greater structural homology with individual groups of HEAT domains than some of the HEAT domain groups have among each other.
Polyglutamine Tract Expansion Increases Protein S-Nitrosylation and the Budding Yeast Zygote Transcriptome
This work has shown that Huntingtin is regulated by posttranslational modifications vii and its role in Huntington’s disease is poorly understood.
The Drosophila Huntington's disease gene ortholog dhtt influences chromatin regulation during development.
Drosophila huntingtin ortholog dhtt modifies phenotypes caused by knockdown of a number of key epigenetic regulators, including chromatin-associated proteins, histone demethylases (HDMs) and methyltransferases, suggesting that a fundamental aspect of huntingtin function in heterochromatin/euchromatin organization is evolutionarily conserved across phyla.
Towards the Development of the TPR Scaffold into Novel Biomaterials & Bioswitches.
The aim is to develop a system in which a ‘switching’ CTPR is used as a sensor that, when triggered by environmental conditions, partially unfolds and oligomerises.
Utilisation des cellules souches embryonnaires humaines porteuses de la mutation causale de la maladie de Huntington en tant que nouveau modèle pathologique
J’ai identifie de nouveaux biomarqueurs cellulaires precoces associes a la mutation causale de la MH, which suggerent l’existence of mecanismes moleculaires encore inconnus et specifiques des phases les plus precoce de the maladie.
Intrinsic disorder in scaffold proteins: getting more from less.
Control of primary metabolism in plants
The Transcriptome and Genome are evaluated to Inform the Study of Metabolic Control in Plants and the Role of Protein Kinases, Phosphatases and 14--3--3 Proteins in the Control of Primary Plant Metabolism is revealed.
Huntingtin gene evolution in Chordata and its peculiar features in the ascidian Ciona genus
During chordate diversification, events of gain/loss, sliding, phase changes, and expansion of introns occurred in both vertebrate and ascidian lineages predominantly in the 5'-half of the htt gene, where there is also evidence of lineage-specific evolutionary dynamics in vertebrates.
Characterization of TOR complex 2 (TORC2) in "Saccharomyces cerevisiae"
It is suggested that TORC2 exists in an oligomeric state and that AVO1 and likely AVO3 act as scaffold proteins required for the integrity ofTORC2, and thatAVO1 plays a role as an adaptor protein mediating efficient phosphorylation of substrates.


Identification of a protein complex that is required for nuclear protein import and mediates docking of import substrate to distinct nucleoporins.
A 9S protein complex from a Xenopus ovary cytosolic subfraction that constitutes this fraction's activity in recognizing a model nuclear import substrate and docking it at the nuclear pore complex is identified and characterized.
Predicting coiled coils from protein sequences
This method was used to delineate coiled-coil domains in otherwise globular proteins, such as the leucine zipper domains in transcriptional regulators, and to predict regions of discontinuity within coiled -coil structures,such as the hinge region in myosin.
Genetics and molecular biology of Huntington's disease
The WW domain: a signalling site in dystrophin?
Hundreds of ankyrin‐like repeats in functionally diverse proteins: Mobile modules that cross phyla horizontally?
Based on pattern searches and systematic database screening, almost 650 different ankyrin‐like (ANK) repeats from nearly all phyla have been identified; more than 150 of them are reported here for
alpha- and beta-forms of the 65-kDa subunit of protein phosphatase 2A have a similar 39 amino acid repeating structure.
The deduced protein sequences of the alpha- and beta-isotypes were found to be made up of 15 imperfect repeating units consisting of 39 amino acids, which was conserved between species.
Nebulin, a helical actin binding protein.
A model for the structure of the F‐actin‐nebulin complex in vivo, based on the assumed importance of charge interactions between nebulin and actin, is proposed and two nebulin molecules occupy symmetrical positions along the central cleft of the actin filament bridging the two strands of theActin two‐start helix.