DecodALS

L’objectif de l’équipe est d’identifier des voies comme cibles thérapeutiques pour ralentir la progression de la maladie par 2 approches :

• Comprendre comment les mutations dans des gènes connus ou à identifier entrainent la mort des motoneurones et le dysfonctionnement de la microglie.
• Découvrir de nouvelles cibles thérapeutiques liées au système immunitaire et à la périphérie (plutôt que dans le système nerveux central (plus facile d’accès par rapport au cerveau) en modulant la réponse des macrophages périphériques à la dégénérescence des motoneurones situés dans la moelle épinière.

Principales publications

2026

• De Bertier S*, Amador MD*, Guissart C, Miki T, Boillée S, Lobsiger CS, Bohl D, Fauret-Amsellem AL, Bohic A; Brainbank Neuro-CEB neuropathology network; Giguet-Valard AG, Bellance R, Beauvais K, Meininger V, Bruneteau G, Salachas F, Vial C, Camu W, Esselin F, de la Cruz E, Bernard E, Seilhean D, Millecamps S. (*equal contributions)
  Familial, neuropathological and cellular analysis identify ARPP21 as a major amyotrophic lateral sclerosis associated gene in French cohorts.
  Acta Neuropathol. 2026 Sep 4;152(1):29. PMID: 42696048
• Miki T, De Bertier S, Amador MD, Nicolas M, Guissart C, Deret M, Teyssou E, Muratet F, Bohl D, Lobsiger CS, Boillée S, Esselin F, Salachas F, Millecamps S*, Seilhean D*. (*equal contributions)
  Neuropathological analysis of an ALS patient carrying a SOD1 missense variant and a C9orf72 repeat expansion.
  Acta Neuropathol. 2026 Apr 17;151(1):40. PMID: 41995858

2025

• Lenoel I, Ribon M, Lorenc F, Diebold A, Philibert CE, Robaldo D, Badsi M, Perronnet J, Lameth J, Berriat F, Misawa H, Coutelier M, Cassel R, Sarrazin N, Jost-Mousseau C, Bohl D, Millecamps S, Mallat M, Brenner D, Weishaupt JH, Boillée S, Lobsiger CS. 
  ALS/FTD-linked TBK1 deficiency in microglia induces an aged-like microglial signature and drives social recognition deficits in mice. 
  Nat Commun. 2025 Aug 26;16(1):7951. PMID: 40858618
• Mei I, Nichterwitz S, Leboeuf M, Nijssen J, Lenoel I, Repsilber D, Lobsiger CS, Hedlund E. 
  Transcriptional modulation unique to vulnerable motor neurons predicts ALS across species and SOD1 mutations.
  Genome Res. 2025 Sep 2;35(9):1975-1991. PMID: 40675818 (collaborative project with Hedlund Team, Karolinska/Stockholm Univ, Sweden)
• De Bertier S, Lautrette G, Amador MD, Miki T, Boillée S, Lobsiger CS, Bohl D, Darios F, Machat S, Duchesne M, Vourc'h P, Fauret-Amsellem AL, Corcia P, Guy N, Couratier P, Seilhean D, Millecamps S.
  MAPT mutations in amyotrophic lateral sclerosis : clinical, neuropathological and functional insights.
  J Neurol. 2025 Mar 18;272(4):272. PMID : 40100285

2024

• Montero AS*, Aliouat I*, Ribon M, Canney M, Goldwirt L, Mourah S, Berriat F, Lobsiger CS, Pradat PF, Salachas F, Bruneteau G, Carpentier A, Boillée S.
  Effect of ultrasound-mediated blood-spinal cord barrier opening on survival and motor function in females in an amyotrophic lateral sclerosis mouse model.
  EBioMedicine. 2024 Aug;106:105235. PMID: 38996764

2023

• Berriat F*, Lobsiger CS*, Boillée S. (*equal contributions)
  The contribution of the peripheral immune system to neurodegeneration.
  Nat Neurosci. 2023 Jun;26(6):942-954. PMID: 37231108 (Review)
• Lefebvre-Omar C, Liu E, Dalle C, d'Incamps BL, Bigou S, Daube C, Karpf L, Davenne M, Robil N, Jost Mousseau C, Blanchard S, Tournaire G, Nicaise C, Salachas F, Lacomblez L, Seilhean D, Lobsiger CS, Millecamps S, Boillée S, Bohl D.
  Neurofilament accumulations in amyotrophic lateral sclerosis patients' motor neurons impair axonal initial segment integrity.
  Cell Mol Life Sci. 2023 May 15;80(6):150. PMID: 37184603
• Megat S, Mora N, Sanogo J, Roman O, Catanese A, Alami NO, Freischmidt A, Mingaj X, De Calbiac H, Muratet F, Dirrig-Grosch S, Dieterle S, Van Bakel N, Müller K, Sieverding K, Weishaupt J, Andersen PM, Weber M, Neuwirth C, Margelisch M, Sommacal A, Van Eijk KR, Veldink JH; Project Mine Als Sequencing Consortium; Lautrette G, Couratier P, Camuzat A, Le Ber I, Grassano M, Chio A, Boeckers T, Ludolph AC, Roselli F, Yilmazer-Hanke D, Millecamps S*, Kabashi E*, Storkebaum E*, Sellier C*, Dupuis L*. (*equal contributions)
  Integrative genetic analysis illuminates ALS heritability and identifies risk genes.
  Nat Commun. 2023 Jan 20;14(1):342. PMID: 36670122 (collaborative project with Dupuis/Kabashi/Storkebaum Teams, FR/NL)

2022

• Teyssou E, Chartier L, Roussel D, Perera ND, Nemazanyy I, Langui D, Albert M, Larmonier T, Saker S, Salachas F, Pradat PF, Meininger V, Ravassard P, Côté F, Lobsiger CS, Boillée S, Turner BJ, Seilhean D, Millecamps S.
  The Amyotrophic Lateral Sclerosis M114T PFN1 Mutation Deregulates Alternative Autophagy Pathways and Mitochondrial Homeostasis.
  Int J Mol Sci. 2022 May 19;23(10):5694. PMID: 35628504

2021

• Muratet F, Teyssou E, Chiot A, Boillée S, Lobsiger CS, Bohl D, Gyorgy B, Guegan J, Marie Y, Amador MDM, Salachas F, Meininger V, Bernard E, Antoine JC, Camdessanché JP, Camu W, Cazeneuve C, Fauret-Amsellem AL, Leguern E, Mouzat K, Guissart C, Lumbroso S, Corcia P, Vourc'h P, Grapperon AM, Attarian S, Verschueren A, Seilhean D, Millecamps S.
  Impact of a frequent nearsplice SOD1 variant in amyotrophic lateral sclerosis: optimising SOD1 genetic screening for gene therapy opportunities.
  J Neurol Neurosurg Psychiatry. 2021 Sep;92(9):942-949. PMID: 33785574
• Ribon M, Leone C, Chiot A, Berriat F, Rampanana M, Cottin J, Bohl D, Millecamps S, Lobsiger CS, Heneka MT, Boillée S.
  Deletion of the inflammatory S100-A9/MRP14 protein does not influence survival in hSOD1G93A ALS mice.
  Neurobiol Aging. 2021 May;101:181-186. PMID: 33626479
• Teyssou E, Muratet F, Amador MD, Ferrien M, Lautrette G, Machat S, Boillée S, Larmonier T, Saker S, Leguern E, Cazeneuve C, Marie Y, Guegan J, Gyorgy B, Cintas P, Meininger V, Le Forestier N, Salachas F, Couratier P, Camu W, Seilhean D, Millecamps S.
  Genetic screening of ANXA11 revealed novel mutations linked to amyotrophic lateral sclerosis.
  Neurobiol Aging. 2021 Mar;99:102.e11-102.e20. PMID: 33218681
• Liu E, Karpf L, Bohl D.
  Neuroinflammation in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia and the Interest of Induced Pluripotent Stem Cells to Study Immune Cells Interactions With Neurons. 
  Front Mol Neurosci. 2021 Dec 14;14:767041. PMID: 34970118 (Review)
• Amador MD, Muratet F, Teyssou E, Boillée S, Millecamps S.
  New advances in Amyotrophic Lateral Sclerosis genetics: Towards gene therapy opportunities for familial and young cases.
  Rev Neurol (Paris). 2021 May;177(5):524-535. PMID: 33810837 (Review)

2020

• Chiot A, Zaïdi S, Iltis C, Ribon M, Berriat F, Schiaffino L, Jolly A, de la Grange P, Mallat M, Bohl D, Millecamps S, Seilhean D, Lobsiger CS, Boillée S.
  Modifying macrophages at the periphery has the capacity to change microglial reactivity and to extend ALS survival.
  Nat Neurosci. 2020 Nov;23(11):1339-1351. PMID: 33077946

2019

• Amador MD, Muratet F, Teyssou E, Banneau G, Danel-Brunaud V, Allart E, Antoine JC, Camdessanché JP, Anheim M, Rudolf G, Tranchant C, Fleury MC, Bernard E, Stevanin G, Millecamps S.
  Spastic paraplegia due to recessive or dominant mutations in ERLIN2 can convert to ALS.
  Neurol Genet. 2019 Nov 13;5(6):e374. PMID: 32042907
• Chiot A, Lobsiger CS, Boillée S.
  New insights on the disease contribution of neuroinflammation in amyotrophic lateral sclerosis.
  Curr Opin Neurol. 2019 Oct;32(5):764-770. PMID: 31306211 (Review)

2017

• Teyssou E*, Chartier L*, Amador MD, Lam R, Lautrette G, Nicol M, Machat S, Da Barroca S, Moigneu C, Mairey M, Larmonier T, Saker S, Dussert C, Forlani S, Fontaine B, Seilhean D, Bohl D, Boillée S, Meininger V, Couratier P, Salachas F, Stevanin G, Millecamps S. (*equal contributions)
  Novel UBQLN2 mutations linked to amyotrophic lateral sclerosis and atypical hereditary spastic paraplegia phenotype through defective HSP70-mediated proteolysis.
  Neurobiol Aging. 2017 Oct;58:239.e11-239.e20. PMID: 28716533

2015

• Mesci P, Zaïdi S, Lobsiger CS, Millecamps S, Escartin C, Seilhean D, Sato H, Mallat M, Boillée S.
  System xC- is a mediator of microglial function and its deletion slows symptoms in amyotrophic lateral sclerosis mice.
  Brain. 2015 Jan;138(Pt 1):53-68. PMID: 25384799