A. Signalisation, 70 5.1.2.2 Activation en, p.72

.. Expression-de-phospho-akt-au-cours-du-développement-rénal, 101 1.3.1 Fin du premier trimestre et début du deuxième trimestre

.. Expression-de-phospho-mtor-au-cours-du-développement-rénal, 106 1.4.1 Fin du premier trimestre et début du deuxième trimestre

.. Expression-de-phospho-mtor, 119 2.1.5 Corrélation entre l'expression de Phospho-AKT et de, p.121

H. Coloration and . Hématoxyline, Eosine, Safran) 1.2.1 Fin du premier trimestre et début du deuxième trimestre

C. Phospho and -. , aucune cellule ne l'exprime au niveau de l'urothélium, contrairement à Phospho-mTOR où seules les cellules parapluies sont positives

. Une-Étude-chromosomique, caryotype : 46,XX) a été complétée par une analyse chromosomique sur puce à ADN mettant en évidence la délétion 17q12 du gène HNF1b

. Néphron, net/fr/sciences- terre-vie/biologie/nephron/ 6. Glomérule rénale. [Image] Disponible le 15/08/2017 sur : https://gecni.medixen.fr/images/glomerule-renale 7. Publication du CUEN. Physiologie et physiopathologie rénales, Disponible le 15, 2017.

L. M. Ernst, E. D. Ruchelli, and D. S. Huff, Color Atlas of Fetal and Neonatal Histology, 2011.
DOI : 10.1007/978-1-4614-0019-6

D. Ulrich, Atlas de poche d'embryologie. Médecine-Sciences Flammarion, 1994.

-. Early, R. D. Khong, and . Malcomson, Keeling's Fetal and neonatal pathology Cinquième édition. 882 p. 13. Mouse kidney development. [Image] Disponible le 15, Renal System Development -Embryology. [Image]. Disponible le 15, 2002.

F. Silva, . Non-neoplastic-kidney, R. Diseases, A. Masyuk, L. Russo et al., American Registry of Pathology Orphanet: Syndrome rein colobome Disponible le 15 Orphanet: Syndrome de Kallmann Disponible le 15/08/2017 sur : http://www.orpha.net/consor Therapeutic Targets In Polycystic Liver Disease Mutations in MKKS cause Bardet-Biedl syndrome Le spectre des maladies par-mutations, Curr Drug Targets BARDET-BIEDL SYNDROME Nat Genet, vol.2126, issue.61, pp.15-21, 2000.

L. Coutte, C. Dreyer, M. Sablin, S. Faivre, and R. E. , PI3K-AKT-mTOR pathway and cancer]. Bull Cancer (Paris), Feb, vol.199, issue.2, pp.173-80, 2012.

T. Brotelle and J. Bay, La voie de signalisation PI3K-AKT-mTOR??: description, d??veloppement th??rapeutique, r??sistances, marqueurs pr??dictifs/pronostiques et applications th??rapeutiques en canc??rologie, Bulletin du Cancer, vol.103, issue.1, 2016.
DOI : 10.1016/j.bulcan.2015.09.011

C. Taha, Z. Liu, J. J. Hasani, H. Sonenberg, N. Klip et al., Opposite Translational Control of GLUT1 and GLUT4 Glucose Transporter mRNAs in Response to Insulin, Journal of Biological Chemistry, vol.269, issue.46, pp.33085-91, 1999.
DOI : 10.1172/JCI115068

J. Brugarolas, Renal-Cell Carcinoma ??? Molecular Pathways and Therapies, New England Journal of Medicine, vol.356, issue.2
DOI : 10.1056/NEJMe068263

D. Gwinn, D. Shackelford, D. Egan, M. Mihaylova, A. Mery et al., AMPK Phosphorylation of Raptor Mediates a Metabolic Checkpoint, Molecular Cell, vol.30, issue.2, pp.214-240, 2008.
DOI : 10.1016/j.molcel.2008.03.003

H. Kim and C. Edelstein, Mammalian target of rapamycin inhibition in polycystic kidney disease: From bench to bedside, Kidney Research and Clinical Practice, vol.31, issue.3, p.132, 2012.
DOI : 10.1016/j.krcp.2012.07.002

F. Dameron, An experimental study of the organogenesis of the lung: the nature and specificity of the epithelio-mesenchymatous interactions], J Embryol Exp Morphol, vol.20, issue.2, pp.151-67, 1968.

C. Dony and P. Gruss, Specific expression of the Hox 1.3 homeo box gene in murine embryonic structures originating from or induced by the mesoderm, EMBO J, vol.6, issue.10, pp.2965-75, 1987.

S. Land, C. Scott, and D. Walker, mTOR signalling, embryogenesis and the control of lung development, Seminars in Cell & Developmental Biology, vol.36, pp.68-78
DOI : 10.1016/j.semcdb.2014.09.023

R. Metzger, O. Klein, G. Martin, and M. Krasnow, The branching programme of mouse lung development, Nature, vol.105, issue.7196, pp.745-50, 2008.
DOI : 10.1038/nature07005

C. Scott, D. Walker, E. Cwiklinski, C. Tait, A. Tee et al., Control of HIF-1?? and vascular signaling in fetal lung involves cross talk between mTORC1 and the FGF-10/FGFR2b/Spry2 airway branching periodicity clock, AJP: Lung Cellular and Molecular Physiology, vol.299, issue.4, pp.455-71, 2010.
DOI : 10.1152/ajplung.00348.2009

L. Kotch, N. Iyer, E. Laughner, and G. Semenza, Defective Vascularization of HIF-1??-Null Embryos Is Not Associated with VEGF Deficiency but with Mesenchymal Cell Death, Developmental Biology, vol.209, issue.2, pp.254-67, 1999.
DOI : 10.1006/dbio.1999.9253

J. Bárdos and M. Ashcroft, Negative and positive regulation of HIF-1: A complex network, Biochimica et Biophysica Acta (BBA) - Reviews on Cancer, vol.1755, issue.2, pp.107-127, 2005.
DOI : 10.1016/j.bbcan.2005.05.001

M. Van-tuyl, J. Liu, J. Wang, M. Kuliszewski, D. Tibboel et al., Role of oxygen and vascular development in epithelial branching morphogenesis of the developing mouse lung, AJP: Lung Cellular and Molecular Physiology, vol.288, issue.1, pp.167-178, 2005.
DOI : 10.1152/ajplung.00185.2004

S. Land, Oxygen-sensing pathways and the development of mammalian gas exchange, Redox Report, vol.2000, issue.1, pp.325-365, 2003.
DOI : 10.1159/000242100

R. Olver, C. Ramsden, L. Strang, and D. Walters, The role of amiloride-blockable sodium transport in adrenaline-induced lung liquid reabsorption in the fetal lamb., The Journal of Physiology, vol.376, issue.1, pp.321-361, 1986.
DOI : 10.1113/jphysiol.1986.sp016156

P. Barker, D. Walters, M. Markiewicz, and L. Strang, Development of the lung liquid reabsorptive mechanism in fetal sheep: synergism of triiodothyronine and hydrocortisone., The Journal of Physiology, vol.433, issue.1, pp.435-484, 1991.
DOI : 10.1113/jphysiol.1991.sp018436

M. Mansley and S. Wilson, Dysregulation of epithelial Na+ absorption induced by inhibition of the kinases TORC1 and TORC2, British Journal of Pharmacology, vol.277, issue.1, 2010.
DOI : 10.1074/jbc.M108951200

S. Wilson, R. Olver, and D. Walters, Developmental regulation of lumenal lung fluid and electrolyte transport, Respiratory Physiology & Neurobiology, vol.159, issue.3, pp.247-55, 2007.
DOI : 10.1016/j.resp.2007.10.004

Y. Wang, L. Zhang, G. Zhou, Z. Liao, H. Ahmad et al., Dietary l-arginine supplementation improves the intestinal development through increasing mucosal Akt and mammalian target of rapamycin signals in intra-uterine growth retarded piglets, British Journal of Nutrition, vol.36, issue.08, pp.1371-81, 2012.
DOI : 10.1203/00006450-199407001-00013

O. Connor, P. Kimball, S. Suryawan, A. Bush, J. Nguyen et al., Regulation of translation initiation by insulin and amino acids in skeletal muscle of neonatal pigs, American Journal of Physiology - Endocrinology And Metabolism, vol.285, issue.1, pp.40-53, 2003.
DOI : 10.1152/ajpendo.00563.2002

M. Rhoads, J. Wu, and G. , Glutamine, arginine, and leucine signaling in the intestine, Amino Acids, vol.271, issue.Suppl, pp.111-133, 2009.
DOI : 10.1042/bj3360001

S. Kim, R. Mcpherson, and G. Wu, Dietary arginine supplementation enhances the growth of milk-fed young pigs, J Nutr, vol.134, issue.3, pp.625-655, 2004.

T. Franke, D. Kaplan, and L. Cantley, PI3K: Downstream AKTion Blocks Apoptosis, Cell, vol.88, issue.4, pp.435-442, 1997.
DOI : 10.1016/S0092-8674(00)81883-8

URL : http://doi.org/10.1016/s0092-8674(00)81883-8

F. Belibi and C. Edelstein, Novel targets for the treatment of autosomal dominant polycystic kidney disease, Expert Opinion on Investigational Drugs, vol.280, issue.3, pp.315-343, 2010.
DOI : 10.1038/ki.1996.383

J. Lopiccolo, G. Blumenthal, W. Bernstein, P. Dennis, G. Hudes et al., Targeting the PI3K/Akt/mTOR pathway: Effective combinations and clinical considerations, Drug Resistance Updates, vol.11, issue.1-2, p.32, 2008.
DOI : 10.1016/j.drup.2007.11.003

. Temsirolimus, or both for advanced renal-cell carcinoma, N Engl J Med, vol.356, issue.22, pp.2271-81, 2007.

M. Holz, The role of S6K1 in ER-positive breast cancer. Cell Cycle, pp.3159-65, 2012.

J. Beaver and B. Park, The BOLERO-2 trial: the addition of everolimus to exemestane in the treatment of postmenopausal hormone receptor-positive advanced breast cancer, Future Oncology, vol.9, issue.6, pp.651-658
DOI : 10.1093/jnci/djk020

J. Tang, Q. He, R. Guo, and X. Chang, Phosphorylated Akt overexpression and loss of PTEN expression in non-small cell lung cancer confers poor prognosis. Lung Cancer Amst Neth, Feb, vol.51, issue.2, pp.181-91, 2006.

R. Maud-anne, Étude du profil mutationnel K-RAS, EGFR, LKB1 et de la transition épithélio-mésenchymateuse dans une série de 22 carcinomes sarcomatoïdes pulmonaires, Thèse de doctorat : médecine. Grenoble : Université Joseph Fourier. Disponible le 15, 2011.

M. Murakami, T. Ichisaka, M. Maeda, N. Oshiro, K. Hara et al., mTOR Is Essential for Growth and Proliferation in Early Mouse Embryos and Embryonic Stem Cells, Molecular and Cellular Biology, vol.24, issue.15, pp.6710-6718, 2004.
DOI : 10.1128/MCB.24.15.6710-6718.2004

URL : http://mcb.asm.org/content/24/15/6710.full.pdf

A. Lan and J. Du, Potential role of Akt signaling in chronic kidney disease. Nephrol Dial Transplant Off Publ Eur Dial Transpl Assoc -Eur Ren Assoc, 2015.

A. Pobuda, Nuclear localized Akt enhances breast cancer stem-like cells through counter-regulation of p21(Waf1/Cip1) and p27(kip1), Cell Cycle Georget Tex, vol.14, issue.13, pp.2109-2129, 2015.

K. Hentges, B. Sirry, A. Gingeras, D. Sarbassov, N. Sonenberg et al., FRAP/mTOR is required for proliferation and patterning during embryonic development in the mouse, Proceedings of the National Academy of Sciences, vol.97, issue.26, pp.13796-801, 2001.
DOI : 10.1073/pnas.011511898

F. Costantini and . Gdnf, GDNF/Ret signaling and renal branching morphogenesis, Organogenesis, vol.129, issue.4, 2010.
DOI : 10.1016/j.ydbio.2007.08.021

URL : http://doi.org/10.1016/j.ydbio.2007.03.091

C. Porta and R. Figlin, Phosphatidylinositol-3-Kinase/Akt Signaling Pathway and Kidney Cancer, and the Therapeutic Potential of Phosphatidylinositol-3-Kinase/Akt Inhibitors, The Journal of Urology, vol.182, issue.6, pp.2569-77, 2009.
DOI : 10.1016/j.juro.2009.08.085

M. Boca, G. Distefano, A. Boletta, F. Qian, A. Bhunia et al., Polycystin-1 Induces Resistance to Apoptosis through the Phosphatidylinositol 3-Kinase/Akt Signaling Pathway, Journal of the American Society of Nephrology, vol.17, issue.3, pp.637-684, 2006.
DOI : 10.1681/ASN.2005050534

M. Hager, H. Haufe, R. Kemmerling, W. Hitzl, G. Mikuz et al., Increased activated Akt expression in renal cell carcinomas and prognosis, Journal of Cellular and Molecular Medicine, vol.5, issue.8b, pp.2181-2189, 2009.
DOI : 10.3816/CGC.2007.n.020

URL : http://onlinelibrary.wiley.com/doi/10.1111/j.1582-4934.2008.00488.x/pdf

A. Skolarikos, A. Papatsoris, G. Alivizatos, and C. Deliveliotis, Molecular Pathogenetics of Renal Cancer, American Journal of Nephrology, vol.26, issue.3, pp.218-249, 2006.
DOI : 10.1159/000093631

D. Cristofano, C. Minervini, A. Menicagli, M. Salinitri, G. Bertacca et al., Nuclear expression of hypoxia-inducible factor-1alpha in clear cell renal cell carcinoma is involved in tumor progression, Am J Surg Pathol, 2007.

D. Guertin and D. Sabatini, Defining the role of mTOR in cancer. Cancer Cell, pp.9-22, 2007.

T. Hartman, E. Nicolas, A. Klein-szanto, T. Saleem, T. Cash et al., The role of the Birt???Hogg???Dub?? protein in mTOR activation and renal tumorigenesis, Oncogene, vol.11, issue.13, pp.1594-604, 2009.
DOI : 10.1038/ncb999

S. Pal, T. Choueiri, K. Wang, D. Khaira, J. Karam et al., Characterization of Clinical Cases of Collecting Duct Carcinoma of the Kidney Assessed by Comprehensive Genomic Profiling, European Urology, vol.70, issue.3, pp.516-537, 2016.
DOI : 10.1016/j.eururo.2015.06.019

J. Wang, A. Papanicolau-sengos, S. Chintala, L. Wei, B. Liu et al., Collecting duct carcinoma of the kidney is associated with <i>CDKN2A</i> deletion and <i>SLC</i> family gene up-regulation, Oncotarget, vol.7, issue.21, pp.29901-29916, 2016.
DOI : 10.18632/oncotarget.9093

D. Anglicheau, N. Pallet, D. Joly, E. Ramos, M. Hoffman et al., Développements actuels des inhibiteurs de mTOR en néphrologie et en transplantation rénale Disponible le 15/08/2017 sur : http://www.sfndt.org/sn Traditional and targeted exome sequencing reveals common, rare and novel functional deleterious variants in RET-signaling complex in a cohort of living US patients with urinary tract malformations, Hum Genet, vol.131, issue.11, pp.1725-1763, 2007.

J. Kissane, Renal cysts in pediatric patients. A classification and overview
DOI : 10.1007/bf00858446

C. Jeanpierre, G. Macé, M. Parisot, V. Morinière, A. Pawtowsky et al., RET and GDNF mutations are rare in fetuses with renal agenesis or other severe kidney development defects, Journal of Medical Genetics, vol.48, issue.7, pp.497-504, 2011.
DOI : 10.1136/jmg.2010.088526

URL : https://hal.archives-ouvertes.fr/hal-00622876

M. Skinner, S. Safford, J. Reeves, M. Jackson, and A. Freemerman, Renal Aplasia in Humans Is Associated with RET Mutations, The American Journal of Human Genetics, vol.82, issue.2, 2008.
DOI : 10.1016/j.ajhg.2007.10.008

URL : http://doi.org/10.1016/j.ajhg.2007.10.008

S. Jain, The many faces of RET dysfunction in kidney, Organogenesis, vol.106, issue.4, pp.177-90, 2009.
DOI : 10.1016/S0092-8674(01)00422-6

I. Yosypiv, Congenital Anomalies of the Kidney and Urinary Tract: A Genetic Disorder?, International Journal of Nephrology, vol.12, issue.11
DOI : 10.1038/nrg3031

M. Consugar, A. Chapman, V. Torres, L. Guay-woodford, and J. Grantham, Comprehensive molecular diagnostics in autosomal dominant polycystic kidney disease, Disponible le 15, pp.2143-60, 2007.

E. Kleymenova, O. Ibraghimov-beskrovnaya, H. Kugoh, J. Everitt, H. Xu et al., Tuberin-Dependent Membrane Localization of Polycystin-1, Molecular Cell, vol.7, issue.4, pp.823-855, 2001.
DOI : 10.1016/S1097-2765(01)00226-X

URL : http://doi.org/10.1016/s1097-2765(01)00226-x

J. Zhou, J. Brugarolas, and L. Parada, Loss of Tsc1, but not Pten, in renal tubular cells causes polycystic kidney disease by activating mTORC1, Human Molecular Genetics, vol.18, issue.22, 2009.
DOI : 10.1093/hmg/ddp398

J. Shillingford, K. Piontek, G. Germino, and T. Weimbs, Rapamycin Ameliorates PKD Resulting from Conditional Inactivation of Pkd1, Journal of the American Society of Nephrology, vol.21, issue.3, 2010.
DOI : 10.1681/ASN.2009040421

URL : http://jasn.asnjournals.org/content/21/3/489.full.pdf

I. Zafar, K. Ravichandran, F. Belibi, R. Doctor, and C. Edelstein, Sirolimus attenuates disease progression in an orthologous mouse model of human autosomal dominant polycystic kidney disease, Kidney International, vol.78, issue.8, pp.754-61, 2010.
DOI : 10.1038/ki.2010.250

G. Walz, Therapeutic approaches in autosomal dominant polycystic kidney disease (ADPKD): is there light at the end of the tunnel? Nephrol Dial Transplant, pp.1752-1759, 2006.

A. Serra, D. Poster, A. Kistler, F. Krauer, R. S. Young et al., Sirolimus and Kidney Growth in Autosomal Dominant Polycystic Kidney Disease, New England Journal of Medicine, vol.363, issue.9, pp.820-829, 2010.
DOI : 10.1056/NEJMoa0907419

URL : http://www.zora.uzh.ch/id/eprint/34854/4/Sirolimus_and_kidney_growth_in_autosomal_dominant_polycystic_2010.pdf

M. Wu, P. Wahl, L. Hir, M. Wackerle-men, Y. Wuthrich et al., Everolimus Retards Cyst Growth and Preserves Kidney Function in a Rodent Model for Polycystic Kidney Disease, Kidney and Blood Pressure Research, vol.30, issue.4, pp.253-262, 2007.
DOI : 10.1159/000104818

D. Fischer, U. Jacoby, L. Pape, C. Ward, E. Kuwertz-broeking et al., Activation of the AKT/mTOR pathway in autosomal recessive polycystic kidney disease (ARPKD) Nephrol Dial Transplant Off Publ Eur Dial Transpl Assoc -Eur Ren Assoc, pp.1819-1846, 2009.

G. Warner, K. Hein, V. Nin, M. Edwards, C. Chini et al., Food Restriction Ameliorates the Development of Polycystic Kidney Disease, Journal of the American Society of Nephrology, vol.27, issue.5, pp.1437-1484, 2016.
DOI : 10.1681/ASN.2015020132

M. Cardenas-rodriguez, F. Irigoin, D. Osborn, C. Gascue, N. Katsanis et al., The Bardet???Biedl syndrome-related protein CCDC28B modulates mTORC2 function and interacts with SIN1 to control cilia length independently of the mTOR complex, Human Molecular Genetics, vol.22, issue.20, pp.4031-4073, 2013.
DOI : 10.1093/hmg/ddt253

D. Yu, S. Guo, Y. Jing, Y. Dong, and L. Wei, A review on hepatocyte nuclear factor-1beta and tumor, Cell & Bioscience, vol.119, issue.7, p.58, 2015.
DOI : 10.1172/JCI37028

URL : http://doi.org/10.1186/s13578-015-0049-3

K. Nakanishi and N. Yoshikawa, Genetic disorders of human congenital anomalies of the kidney and urinary tract (CAKUT). Pediatr Int Off J Jpn Pediatr Soc, 2003.