A human skeletal muscle interactome centered on proteins involved in muscular dystrophies: LGMD interactome.

Gaëlle Blandin 1 Sylvie Marchand 1 Karine Charton 1 Nathalie Danièle 1 Evelyne Gicquel 1 Jean-Baptiste Boucheteil 1 Azéddine Bentaib 1 Laetitia Barrault 1 Daniel Stockholm 1 Marc Bartoli 1 Isabelle Richard 1, *
* Auteur correspondant
1 LAMBE - UMR 8587 - Laboratoire Analyse et Modélisation pour la Biologie et l'Environnement
Abstract : ABSTRACT: BACKGROUND: The complexity of the skeletal muscle and the identification of numerous human disease-causing mutations in its constitutive proteins make it an interesting tissue for proteomic studies aimed at understanding functional relationships of interacting proteins in both health and diseases. METHOD: We undertook a large-scale study using two-hybrid screens and a human skeletal-muscle cDNA library to establish a proteome-scale map of protein-protein interactions centered on proteins involved in limb-girdle muscular dystrophies (LGMD). LGMD is a group of more than 20 different neuromuscular disorders that principally affect the proximal pelvic and shoulder girdle muscles.Results and conclusion: The interaction network we unraveled incorporates 1018 proteins connected by 1492 direct binary interactions and includes 1420 novel protein-protein interactions. Computational, experimental and literature-based analyses were performed to assess the overall quality of this network. Interestingly, LGMD proteins were shown to be highly interconnected, in particular indirectly through sarcomeric proteins. In-depth mining of the LGMD-centered interactome identified new candidate genes for orphan LGMDs and other neuromuscular disorders. The data also suggest the existence of functional links between LGMD2B/dysferlin and gene regulation, between LGMD2C/gamma-sarcoglycan and energy control and between LGMD2G/telethonin and maintenance of genome integrity. This dataset represents a valuable resource for future functional investigations.
Keywords : Interactome Muscular dystrophies Yeast-two hybrid
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Article dans une revue
Skeletal Muscle, BioMed Central, 2013, 3 (1), pp.3. 〈10.1186/2044-5040-3-3〉
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Gaëlle Blandin, Sylvie Marchand, Karine Charton, Nathalie Danièle, Evelyne Gicquel, et al.. A human skeletal muscle interactome centered on proteins involved in muscular dystrophies: LGMD interactome.. Skeletal Muscle, BioMed Central, 2013, 3 (1), pp.3. 〈10.1186/2044-5040-3-3〉. 〈inserm-00805816〉

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