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Bienvenu(e) sur le site de l'Institut Necker Enfants Malades
Mercredi 8 juillet 2020
A collaboration between the Pende, Terzi and Pontoglio groups reveals pathological mechanisms in polycystic kidneys.

Polycystic kidney is a debilitating condition observed in several genetic diseases, including Ciliopathies, Autosomal Dominant Polycystic Kidney Disease (ADPKD) and Tuberous Sclerosis Complex. This disorder is accompanied by the activation of the mammalian Target Of Rapamycin (mTOR) pathway, a master regulator of the growth responses within the cell. In a Nature Communications article published in June 2020, three research groups at Inem shed new light on how mTOR may lead to polycystic kidney. Using cellular and animal models of Tuberous Sclerosis Complex, the Pende group in collaboration with the Terzi and Pontoglio teams demonstrates that mTOR not only promotes cell growth and proliferation but also regulates oriented cell division. They provide evidence that, when mTOR activity is uncontrolled, kidney epithelial cells do not proliferate anymore along the tubular axis but in a distorted way leading to tubule enlargement rather than elongation. The molecular players may become new therapeutic targets.   


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Parrain(s)
S.A.R. la Princesse Caroline de Hanovre qui, à travers la Fondation Princesse Grace, soutient déjà la recherche medicale et tout ce qui contribue à soulager les enfants malades en France et dans le monde, a accepté de s'engager à nos cotés pour que ce centre de medecine moleculaire, tourné entre autres vers les pathologies des enfants, prenne de vitesse les maladies et continue à relever les defis actuels.

INEM - Organigramme