Research Team

Molecular Pathophysiology of Adrenal and Endocrine Tissues

HomeTeamsMolecular Pathophysiology of Adrenal and Endocrine Tissues
Our team is interested in the pathophysiology of the adrenal cortex and endocrine tissues. We focus on the molecular mechanisms involved in adrenal and gonads differentiation and how their deregulation results in tumorigenesis and disease. Our team is internationally recognized for its expertise on transgenic mouse models of adrenal and gonadal diseases.


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49 publications

Loss of SUMO-specific protease 2 causes isolated glucocorticoid deficiency by blocking adrenal cortex zonal transdifferentiation in mice.

Published on 21 Dec 2022 in Nature communications , vol. 13 - pp 7858

Dufour D , Dumontet T , Sahut-Barnola I, Carusi A, Onzon M , Pussard E, Wilmouth JJ , Olabe J , Lucas C , Levasseur A , Damon-Soubeyrand C , Pointud JC , Roucher-Boulez F , Tauveron I , Bossis G, Yeh ET, Breault DT, Val P , Lefrançois-Martinez AM, Martinez A


Loss of LGR4/GPR48 causes severe neonatal salt-wasting due to disrupted WNT signaling altering adrenal zonation.

Published on 20 Dec 2022 in The Journal of clinical investigation

Lucas C , Sauter KS, Steigert M, Mallet D, Wilmouth J Jr , Olabe J , Plotton I, Morel Y, Aeberli D, Wagner F, Clevers H, Pandey AV, Val P , Roucher-Boulez F , Fluck CE


Steroidogenic Factor-1 Lineage Origin of Skin Lesions in Carney Complex Syndrome.

Published on 12 Nov 2022 in The Journal of investigative dermatology , vol. 142 - pp 2949-2957.e9

Sahut-Barnola I , Lefrançois-Martinez AM , Dufour D , Botto JM, Kamilaris C, Faucz FR, Stratakis CA, Val P , Martinez A


β-Catenin activation and illicit receptor expression in adrenocortical cells.

Published on 07 Feb 2022 in Endocrine-related cancer , vol. 29 - pp 151-162

Le Mestre J, Thomas M, Duparc C, Val P , Bures C, Tuech JJ, Sabourin JC, Baert-Desurmont S, Lefebvre H, Louiset E


Protein kinase A drives paracrine crisis and WNT4-dependent testis tumor in Carney complex.

Published on 01 Dec 2021 in The Journal of clinical investigation , vol. 131

Djari C , Sahut-Barnola I , Septier A , Plotton I, Montanier N , Dufour D , Levasseur A , Wilmouth J Jr , Pointud JC , Faucz FR, Kamilaris C, Lopez AG, Guillou F, Swain A, Vainio SJ, Tauveron I , Val P , Lefebvre H, Stratakis CA, Martinez A , Lefrançois-Martinez AM


How can we minimise the use of regular oral corticosteroids in asthma?

Published on 31 Mar 2020 in European respiratory review : an official journal of the European Respiratory Society , vol. 29

Bourdin A, Adcock I, Berger P, Bonniaud P, Chanson P, Chenivesse C, de Blic J, Deschildre A, Devillier P, Devouassoux G, Didier A, Garcia G, Magnan A, Martinat Y, Perez T, Roche N, Taillé C, Val P , Chanez P


Hormonal and spatial control of SUMOylation in the human and mouse adrenal cortex.

Published on 12 Sep 2019 in FASEB journal : official publication of the Federation of American Societies for Experimental Biology , vol. 33 - pp 10218-10230

Dumontet T , Sahut-Barnola I , Dufour D , Lefrançois-Martinez AM , Berthon A , Montanier N , Ragazzon B, Djari C , Pointud JC , Roucher-Boulez F , Batisse-Lignier M , Tauveron I , Bertherat J, Val P , Martinez A


A ZNRF3-dependent Wnt/β-catenin signaling gradient is required for adrenal homeostasis.

Published on 01 Feb 2019 in Genes & development , vol. 33 - pp 209-220

Basham KJ, Rodriguez S , Turcu AF, Lerario AM, Logan CY, Rysztak MR, Gomez-Sanchez CE, Breault DT, Koo BK, Clevers H, Nusse R, Val P , Hammer GD


PKA regulatory subunit 1A inactivating mutation induces serotonin signaling in primary pigmented nodular adrenal disease.

Published on 22 Sep 2016 in JCI insight , vol. 1 - pp e87958

Bram Z, Louiset E, Ragazzon B, Renouf S, Wils J, Duparc C, Boutelet I, Rizk-Rabin M, Libé R, Young J, Carson D, Vantyghem MC, Szarek E, Martinez A , Stratakis CA, Bertherat J, Lefebvre H


EZH2 is overexpressed in adrenocortical carcinoma and is associated with disease progression.

Published on 01 Jul 2016 in Human molecular genetics , vol. 25 - pp 2789-2800

Drelon C, Berthon A, Mathieu M, Ragazzon B, Kuick R, Tabbal H, Septier A, Rodriguez S, Batisse-Lignier M, Sahut-Barnola I, Dumontet T, Pointud JC, Lefrançois-Martinez AM, Baron S , Giordano TJ, Bertherat J, Martinez A, Val P


The adrenal capsule is a signaling center controlling cell renewal and zonation through Rspo3.

Published on 15 Jun 2016 in Genes & development , vol. 30 - pp 1389-94

Vidal V, Sacco S, Rocha AS, da Silva F, Panzolini C, Dumontet T , Doan TM, Shan J, Rak-Raszewska A, Bird T, Vainio S, Martinez A , Schedl A


Editorial: Adrenal Cortex: From Physiology to Disease.

Published on 01 Jun 2016 in Frontiers in endocrinology , vol. 7 - pp 51

Val P , Martinez A


Pregnancy, Primary Aldosteronism, and Somatic CTNNB1 Mutations.

Published on 14 Apr 2016 in The New England journal of medicine , vol. 374 - pp 1493-4

Berthon A , Drelon C , Val P


Combined transcriptome studies identify AFF3 as a mediator of the oncogenic effects of β-catenin in adrenocortical carcinoma.

Published on 27 Jul 2015 in Oncogenesis , vol. 4 - pp e161

Lefèvre L, Omeiri H, Drougat L, Hantel C, Giraud M, Val P , Rodriguez S , Perlemoine K, Blugeon C, Beuschlein F, de Reyniès A, Rizk-Rabin M, Bertherat J, Ragazzon B


Adrenal cortex tissue homeostasis and zonation: A WNT perspective.

Published on 15 Jun 2015 in Molecular and cellular endocrinology , vol. 408 - pp 156-64

Drelon C , Berthon A , Mathieu M , Martinez A , Val P


Evaluation of atorvastatin efficacy and toxicity on spermatozoa, accessory glands and gonadal hormones of healthy men: a pilot prospective clinical trial.

Published on 12 Jul 2014 in Reproductive biology and endocrinology : RB&E , vol. 12 - pp 65

Pons-Rejraji H , Brugnon F , Sion B, Maqdasy S , Gouby G, Pereira B, Marceau G , Gremeau AS, Drevet J , Grizard G, Janny L , Tauveron I

WNT/β-catenin signalling is activated in aldosterone-producing adenomas and controls aldosterone production.

Published on 15 Feb 2014 in Human molecular genetics , vol. 23 - pp 889-905

Berthon A , Drelon C , Ragazzon B, Boulkroun S, Tissier F, Amar L, Samson-Couterie B, Zennaro MC, Plouin PF, Skah S, Plateroti M, Lefèbvre H, Sahut-Barnola I , Batisse-Lignier M , Assié G, Lefrançois-Martinez AM , Bertherat J, Martinez A , Val P


WT1 maintains adrenal-gonadal primordium identity and marks a population of AGP-like progenitors within the adrenal gland.

Published on 14 Oct 2013 in Developmental cell , vol. 27 - pp 5-18

Bandiera R, Vidal VP, Motamedi FJ, Clarkson M, Sahut-Barnola I , von Gise A, Pu WT, Hohenstein P, Martinez A , Schedl A


Hypothalamo-pituitary and immune-dependent adrenal regulation during systemic inflammation.

Published on 03 Sep 2013 in Proceedings of the National Academy of Sciences of the United States of America , vol. 110 - pp 14801-6

Kanczkowski W, Alexaki VI, Tran N, Großklaus S, Zacharowski K, Martinez A , Popovics P, Block NL, Chavakis T, Schally AV, Bornstein SR


Adrenocortical cancer and IGF2: is the game over or our experimental models limited?

Published on 12 Feb 2013 in The Journal of clinical endocrinology and metabolism , vol. 98 - pp 505-7

Drelon C , Berthon A , Val P


In vivo evidence for the crucial role of SF1 in steroid-producing cells of the testis, ovary and adrenal gland.

Published on 12 Dec 2012 in Development (Cambridge, England) , vol. 139 - pp 4561-70

Buaas FW, Gardiner JR, Clayton S, Val P , Swain A


Depressed levels of prostaglandin F2α in mice lacking Akr1b7 increase basal adiposity and predispose to diet-induced obesity.

Published on 30 Nov 2012 in Diabetes , vol. 61 - pp 2796-806

Volat FE, Pointud JC , Pastel E , Morio B, Sion B, Hamard G, Guichardant M, Colas R, Lefrançois-Martinez AM , Martinez A

Analysis of the role of Igf2 in adrenal tumour development in transgenic mouse models.

Published on 28 Aug 2012 in PloS one , vol. 7 - pp e44171

Drelon C , Berthon A , Ragazzon B, Tissier F, Bandiera R, Sahut-Barnola I, de Joussineau C , Batisse-Lignier M , Lefrançois-Martinez AM, Bertherat J, Martinez A , Val P


Aldo-Keto Reductases 1B in Endocrinology and Metabolism.

Published on 02 Aug 2012 in Frontiers in pharmacology , vol. 3 - pp 148

Pastel E , Pointud JC , Volat F, Martinez A , Lefrançois-Martinez AM

Wnt/β-catenin signalling in adrenal physiology and tumour development.

Published on 31 Mar 2012 in Molecular and cellular endocrinology , vol. 351 - pp 87-95

Berthon A , Martinez A , Bertherat J, Val P


The cAMP pathway and the control of adrenocortical development and growth.

Published on 31 Mar 2012 in Molecular and cellular endocrinology , vol. 351 - pp 28-36

de Joussineau C , Sahut-Barnola I , Levy I, Saloustros E, Val P , Stratakis CA, Martinez A


Gene dosage effects and transcriptional regulation of early mammalian adrenal cortex development.

Published on 08 Jul 2010 in Molecular and cellular endocrinology , vol. 323 - pp 105-14

Val P , Swain A


Cushing’s syndrome and fetal features resurgence in adrenal cortex-specific Prkar1a knockout mice.

Published on 10 Jun 2010 in PLoS genetics , vol. 6 - pp e1000980

Sahut-Barnola I , de Joussineau C , Val P , Lambert-Langlais S, Damon C, Lefrançois-Martinez AM, Pointud JC , Marceau G , Sapin V , Tissier F, Ragazzon B, Bertherat J, Kirschner LS, Stratakis CA, Martinez A


Constitutive beta-catenin activation induces adrenal hyperplasia and promotes adrenal cancer development.

Published on 15 Apr 2010 in Human molecular genetics , vol. 19 - pp 1561-76

Berthon A , Sahut-Barnola I , Lambert-Langlais S, de Joussineau C , Damon-Soubeyrand C , Louiset E, Taketo MM, Tissier F, Bertherat J, Lefrançois-Martinez AM, Martinez A , Val P


Pasture-feeding of Charolais steers influences skeletal muscle metabolism and gene expression.

Published on 30 Oct 2009 in Journal of physiology and pharmacology : an official journal of the Polish Physiological Society , vol. 60 Suppl 3 - pp 83-90

Cassar-Malek I, Jurie C, Bernard C, Barnola I , Micol D, Hocquette JF

Aldo keto reductase 1B7 and prostaglandin F2alpha are regulators of adrenal endocrine functions.

Published on 07 Oct 2009 in PloS one , vol. 4 - pp e7309

Lambert-Langlais S, Pointud JC , Lefrançois-Martinez AM , Volat F, Manin M, Coudoré F, Val P , Sahut-Barnola I , Ragazzon B, Louiset E, Delarue C, Lefebvre H, Urade Y, Martinez A


Inactivation of the Carney complex gene 1 (protein kinase A regulatory subunit 1A) inhibits SMAD3 expression and TGF beta-stimulated apoptosis in adrenocortical cells.

Published on 15 Sep 2009 in Cancer research , vol. 69 - pp 7278-84

Ragazzon B, Cazabat L, Rizk-Rabin M, Assie G, Groussin L, Fierrard H, Perlemoine K, Martinez A , Bertherat J

The transcription co-factor CITED2 functions during sex determination and early gonad development.

Published on 15 Aug 2009 in Human molecular genetics , vol. 18 - pp 2989-3001

Buaas FW, Val P , Swain A


Mouse model for bilateral adrenal hyperplasia.

Published on 30 Jun 2009 in Annales d'endocrinologie , vol. 70 - pp 194

Sahut-Barnola I , De Joussineau C , Val P , Lambert-Langlais S, Lefrançois-Martinez AM , Pointud JC , Marceau G , Sapin V , Ragazzon B, Bertherat J, Kirschner LS, Stratakis CA, Martinez A


A transgenic mouse line with specific Cre recombinase expression in the adrenal cortex.

Published on 05 Mar 2009 in Molecular and cellular endocrinology , vol. 300 - pp 197-204

Lambert-Langlais S, Val P , Guyot S, Ragazzon B, Sahut-Barnola I , De Haze A , Lefrançois-Martinez AM , Martinez A


Prostaglandin F2alpha synthase activities of aldo-keto reductase 1B1, 1B3 and 1B7.

Published on 02 Mar 2009 in Journal of biochemistry , vol. 145 - pp 161-8

Kabututu Z, Manin M, Pointud JC , Maruyama T, Nagata N, Lambert S , Lefrançois-Martinez AM, Martinez A , Urade Y


Oryza Tag Line, a phenotypic mutant database for the Genoplante rice insertion line library.

Published on 30 Jan 2008 in Nucleic acids research , vol. 36 - pp D1022-7

Larmande P, Gay C, Lorieux M, Périn C, Bouniol M, Droc G, Sallaud C, Perez P, Barnola I , Biderre-Petit C, Martin J, Morel JB, Johnson AA, Bourgis F, Ghesquière A, Ruiz M, Courtois B, Guiderdoni E


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James Wilmouth
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Mickael Mathieu
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Annabel Berthon
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