Fiche publication
Date publication
novembre 2014
Journal
Medecine sciences : M/S
Auteurs
Membres identifiés du Cancéropôle Est :
Dr POCH Olivier
Tous les auteurs :
Chennen K, Scerbo MJ, Dollfus H, Poch O, Marion V
Lien Pubmed
Résumé
The primary cilium is a specialized organelle, present at the surface of most eukaryotic cells, whose main function is to detect, integrate and transmit intra- and extra-cellular signals. Its dysfunction usually results in a group of severe clinical manifestations nowadays termed ciliopathies. The latter can be of syndromic nature with multi-organ dysfunctions and can also be associated with a morbid obese phenotype, like it is the case in the iconic ciliopathy, the Bardet Biedl syndrome (BBS). This review will discuss the contribution of the unique context offered by the emblematic BBS for understanding the mechanisms leading to obesity via the involvement of the primary cilium together with identification of novel molecular players and signaling pathways it has helped to highlight. In the current context of translational medicine and system biology, this article will also discuss the potential benefits and challenges posed by these techniques via multi-level approaches to better dissect the underlying mechanisms leading to the complex condition of obesity.
Mots clés
Adipose Tissue, pathology, Animals, Bardet-Biedl Syndrome, genetics, Bone Marrow, pathology, Chaperonins, deficiency, Cilia, physiology, Ciliary Motility Disorders, genetics, Disease Models, Animal, Endocrine System, physiopathology, Genes, Recessive, Humans, Hypothalamus, physiopathology, Mice, Mice, Knockout, Microtubule-Associated Proteins, deficiency, Models, Biological, Nerve Tissue Proteins, deficiency, Obesity, genetics, Signal Transduction, genetics, Syndrome, Weight Gain
Référence
Med Sci (Paris). 2014 Nov;30(11):1034-9
