Journal of
Medical Genetics and Genomics

  • Abbreviation: J. Med. Genet. Genomics
  • Language: English
  • ISSN: 2141-2278
  • DOI: 10.5897/JMGG
  • Start Year: 2009
  • Published Articles: 48

Full Length Research Paper

Immunodetection of Ribin-like proteins in neuron-based cellular models

Roxane-Marie Barthélémy1*, Nadia Jaafari2, Pascale Galea3, Yvon Julé4 and Eric Faure1
1LATP, UMR6632, Evolution biologique et modélisation, case 18, Université de Provence, 3 Place Victor Hugo, 13331 Marseille cedex 3, France. 2MRC Centre for Synaptic Plasticity, Department of Anatomy, School of Medical Sciences, University of Bristol, Bristol BS8 1TD, United Kingdom 3Sysdiag, UMR3145 (CNRS/BioRad), Cap Delta, 1682 rue de la Valsière, CS61003, 34164 Montpellier, France. 4Biovays, case 18, Université de Provence, 3 Place Victor Hugo, 13331 Marseille cedex 3, France.
Email: [email protected]

  •  Accepted: 14 July 2010
  •  Published: 30 November 2010

Abstract

Extensive bibliographical analysis has demonstrated changes in ribin genes expression during several types of stress, especially in neurological tissues or cells during stress inducing neurological disorders. These analyses suggest that Ribin studies could be useful for neurological investigation. The present study was undertaken to detect and localize Ribin in various neuron-based cellular models mimicking neurodegenerative pathologies such as Huntington’s disease, amyotropic lateral sclerosis and spinal muscular atrophy, as well as pathologies. Using confocal microscopy immunofluorescence methods, the presence of Ribin was detected in all neuronal models. Change, in the protein level was found at least in one neuronal model, suggesting that this protein could play a physiological role in diseases. Moreover, in contrast to previous experiments, we showed that Ribin-like proteins had principally a cytoplasmic localization. Indeed, BLAST analysis of muridae and human DNA-databases provides evidence that most of the sequence homologies are found within the Ribin COOH-part involved in the loss of the nuclear localization signals, which suggests the synthesis of cytoplasmic Ribin-like proteins.

 

Key words: Ribin, neuron-based cellular models, amyotropic lateral sclerosis, Huntington’s disease, spinal muscular atrophy.