Research Topics
Genomes and Genes | Rajesh P MenonSummaryAffiliation: National Institute for Medical Research Country: UK Publications
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Detail Information
Publications
The solution structure of the Josephin domain of ataxin-3: structural determinants for molecular recognitionGiuseppe Nicastro
National Institute for Medical Research, The Ridgeway, London NW7 1AA, United Kingdom
Proc Natl Acad Sci U S A 102:10493-8. 2005..The structural similarity with other deubiquitinating enzymes suggests a model for the proteolytic enzymatic activity of ataxin-3...
The importance of serine 776 in Ataxin-1 partner selection: a FRET analysisRajesh P Menon
MRC National Institute for Medical Research, The Ridgeway, London NW7 1AA UK
Sci Rep 2:919. 2012..These results indicate that Ser776 acts as the molecular switch that discriminates between normal and aberrant function and that phosphomimetics is not a generally valid approach whose applicability should be carefully validated...
The C terminus of fragile X mental retardation protein interacts with the multi-domain Ran-binding protein in the microtubule-organising centreRajesh P Menon
National Institute for Medical Research, London NW7 1AA, UK
J Mol Biol 343:43-53. 2004..The interaction with FMRP was mapped in a newly identified CRA motif present in the RanBPM C terminus. Our results suggest that the functional role of RanBPM binding is modulation of the RNA-binding properties of FMRP...
Hammerhead ribozyme-mediated silencing of the mutant fibrillin-1 of tight skin mouse: insight into the functional role of mutant fibrillin-1Rajesh P Menon
Centre for Rheumatology, Royal Free and University College Medical School, Division of Medicine, University College London Hampstead Campus, Rowland Hill Street, London NW3 2PF2, UK
Exp Cell Res 312:1463-74. 2006..Our results underline the therapeutic potential of hammerhead ribozymes in dominant negative defects and suggest that changes in microfibril architecture brought about by fibrillin-1 mutation lead to a complex disease phenotype...
Polyglutamine is not all: the functional role of the AXH domain in the ataxin-1 proteinCesira de Chiara
National Institute for Medical Research, The Ridgeway, London NW71AA, UK
J Mol Biol 354:883-93. 2005..These results provide the first direct evidence of an involvement of a region other than the polyglutamine tract in polyglutamine pathologies...
Josephin domain of ataxin-3 contains two distinct ubiquitin-binding sitesGiuseppe Nicastro
National Institute for Medical Research, The Ridgeway, London NW7 1AA, UK
Biopolymers 91:1203-14. 2009..The presence of two ubiquitin-binding sites explains how ataxin-3 binds poly-ubiquitin chains and provides new insights into the molecular mechanism of ubiquitin recognition...
The AXH domain adopts alternative folds the solution structure of HBP1 AXHCesira de Chiara
National Institute for Medical Research, The Ridgeway, London, United Kingdom
Structure 13:743-53. 2005..Our results describe an entirely novel, to our knowledge, example of a chameleon fold and suggest a link between these properties and the SCA1 pathogenesis...
Characterization of the structure and the amyloidogenic properties of the Josephin domain of the polyglutamine-containing protein ataxin-3Laura Masino
National Institute for Medical Research, The Ridgeway, London NW7 1AA, UK
J Mol Biol 344:1021-35. 2004..Our data support a mechanism in which the thermodynamic stability of ataxin-3 is governed by the properties of the Josephin domain, but the presence of an expanded polyQ tract increases dramatically the protein's tendency to aggregate...
Structural bases for recognition of Anp32/LANP proteinsCesira de Chiara
National Institute for Medical Research, The Ridgeway, London, UK
FEBS J 275:2548-60. 2008..e. the microtubule plus-end tracking protein Clip 170/Restin, known to regulate the dynamic properties of microtubules and to be associated with severe human pathologies...
Domain architecture of the polyglutamine protein ataxin-3: a globular domain followed by a flexible tailLaura Masino
National Institute for Medical Research, The Ridgeway, London NW7 1AA, UK
FEBS Lett 549:21-5. 2003..Moreover, [(15)N]glutamine selectively labelled samples allowed us to have a direct insight by NMR into the structure of the polyQ region...
Specificity of interactions of galectin-3 with Chrp, a cysteine- and histidine-rich cytoplasmic proteinSulemana Bawumia
National Institute for Medical Research, Mill Hill, London NW7 1AA, UK
Biochimie 85:189-94. 2003..The formation of higher-order complexes containing these proteins and additional binding partners may be relevant to cytoplasmic functions involving galectin-3...
Down-regulation of the dopamine receptor D2 in mice lacking ataxin 1Robert Goold
UCL Institute of Child Health, University College London, UK
Hum Mol Genet 16:2122-34. 2007..Therefore, this study identifies novel molecular targets that are regulated by Atxn1 which might contribute to the motor deficits in SCA1, and provides new insights into the mechanisms by which Atxn1 co-regulates transcription...
Assignment of the 1H, 13C, and 15N resonances of the Josephin domain of human ataxin-3Giuseppe Nicastro
J Biomol NMR 30:457-8. 2004
