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STAT3 gain of function

MedGen UID:
925793
Concept ID:
C4288261
Cell or Molecular Dysfunction
Synonyms: Activating STAT3 Gene Mutation; Activating STAT3 Mutation; STAT3 Activating Gene Mutation; STAT3 Activating Mutation; STAT3 Gain of Function; STAT3 Gain of Function Gene Mutation; STAT3 Gain of Function Mutation

Definition

A mutation in the STAT3 gene that results in an increase in cytokine-related function. STAT3 mutations of this type are associated with infantile-onset multisystem autoimmune disease. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • STAT3 gain of function

Professional guidelines

PubMed

Moser LM, Fekadu J, Willasch A, Rettinger E, Sörensen J, Jarisch A, Kirwil M, Lieb A, Holzinger D, Calaminus G, Bader P, Bakhtiar S
Br J Haematol 2023 Mar;200(5):595-607. Epub 2022 Oct 10 doi: 10.1111/bjh.18497. PMID: 36214981
Mulvihill C, Fitzgerald K, O'Regan E, Healy CM, Leahy TR
Oral Oncol 2022 Sep;132:106015. Epub 2022 Jul 6 doi: 10.1016/j.oraloncology.2022.106015. PMID: 35809509
Hadjadj J, Frémond ML, Neven B
Front Immunol 2021;12:717388. Epub 2021 Sep 17 doi: 10.3389/fimmu.2021.717388. PMID: 34603291Free PMC Article

Recent clinical studies

Etiology

Atschekzei F, Traidl S, Carlens J, Schütz K, von Hardenberg S, Elsayed A, Ernst D, Risser L, Thiele T, Graalmann T, Raab J, Baumann U, Witte T, Sogkas G
Front Immunol 2024;15:1400348. Epub 2024 Aug 23 doi: 10.3389/fimmu.2024.1400348. PMID: 39247195Free PMC Article
Maccari ME, Wolkewitz M, Schwab C, Lorenzini T, Leiding JW, Aladjdi N, Abolhassani H, Abou-Chahla W, Aiuti A, Azarnoush S, Baris S, Barlogis V, Barzaghi F, Baumann U, Bloomfield M, Bohynikova N, Bodet D, Boutboul D, Bucciol G, Buckland MS, Burns SO, Cancrini C, Cathébras P, Cavazzana M, Cheminant M, Chinello M, Ciznar P, Coulter TI, D'Aveni M, Ekwall O, Eric Z, Eren E, Fasth A, Frange P, Fournier B, Garcia-Prat M, Gardembas M, Geier C, Ghosh S, Goda V, Hammarström L, Hauck F, Heeg M, Heropolitanska-Pliszka E, Hilfanova A, Jolles S, Karakoc-Aydiner E, Kindle GR, Kiykim A, Klemann C, Koletsi P, Koltan S, Kondratenko I, Körholz J, Krüger R, Jeziorski E, Levy R, Le Guenno G, Lefevre G, Lougaris V, Marzollo A, Mahlaoui N, Malphettes M, Meinhardt A, Merlin E, Meyts I, Milota T, Moreira F, Moshous D, Mukhina A, Neth O, Neubert J, Neven B, Nieters A, Nove-Josserand R, Oksenhendler E, Ozen A, Olbrich P, Perlat A, Pac M, Schmid JP, Pacillo L, Parra-Martinez A, Paschenko O, Pellier I, Sefer AP, Plebani A, Plantaz D, Prader S, Raffray L, Ritterbusch H, Riviere JG, Rivalta B, Rusch S, Sakovich I, Savic S, Scheible R, Schleinitz N, Schuetz C, Schulz A, Sediva A, Semeraro M, Sharapova SO, Shcherbina A, Slatter MA, Sogkas G, Soler-Palacin P, Speckmann C, Stephan JL, Suarez F, Tommasini A, Trück J, Uhlmann A, van Aerde KJ, van Montfrans J, von Bernuth H, Warnatz K, Williams T, Worth AJJ, Ip W, Picard C, Catherinot E, Nademi Z, Grimbacher B, Forbes Satter LR, Kracker S, Chandra A, Condliffe AM, Ehl S; European Society for Immunodeficiencies Registry Working Party
J Allergy Clin Immunol 2023 Oct;152(4):984-996.e10. Epub 2023 Jun 28 doi: 10.1016/j.jaci.2023.06.015. PMID: 37390899
Leiding JW, Vogel TP, Santarlas VGJ, Mhaskar R, Smith MR, Carisey A, Vargas-Hernández A, Silva-Carmona M, Heeg M, Rensing-Ehl A, Neven B, Hadjadj J, Hambleton S, Ronan Leahy T, Meesilpavikai K, Cunningham-Rundles C, Dutmer CM, Sharapova SO, Taskinen M, Chua I, Hague R, Klemann C, Kostyuchenko L, Morio T, Thatayatikom A, Ozen A, Scherbina A, Bauer CS, Flanagan SE, Gambineri E, Giovannini-Chami L, Heimall J, Sullivan KE, Allenspach E, Romberg N, Deane SG, Prince BT, Rose MJ, Bohnsack J, Mousallem T, Jesudas R, Santos Vilela MMD, O'Sullivan M, Pachlopnik Schmid J, Průhová Š, Klocperk A, Rees M, Su H, Bahna S, Baris S, Bartnikas LM, Chang Berger A, Briggs TA, Brothers S, Bundy V, Chan AY, Chandrakasan S, Christiansen M, Cole T, Cook MC, Desai MM, Fischer U, Fulcher DA, Gallo S, Gauthier A, Gennery AR, Gonçalo Marques J, Gottrand F, Grimbacher B, Grunebaum E, Haapaniemi E, Hämäläinen S, Heiskanen K, Heiskanen-Kosma T, Hoffman HM, Gonzalez-Granado LI, Guerrerio AL, Kainulainen L, Kumar A, Lawrence MG, Levin C, Martelius T, Neth O, Olbrich P, Palma A, Patel NC, Pozos T, Preece K, Lugo Reyes SO, Russell MA, Schejter Y, Seroogy C, Sinclair J, Skevofilax E, Suan D, Suez D, Szabolcs P, Velasco H, Warnatz K, Walkovich K, Worth A; STAT3 GOF Working Group members, Seppänen MRJ, Torgerson TR, Sogkas G, Ehl S, Tangye SG, Cooper MA, Milner JD, Forbes Satter LR
J Allergy Clin Immunol 2023 Apr;151(4):1081-1095. Epub 2022 Oct 11 doi: 10.1016/j.jaci.2022.09.002. PMID: 36228738Free PMC Article
López AL, Niemela J, Stoddard J, Paolini MV, Rosenzweig S, Fernández Romero DS
Medicina (B Aires) 2021;81(6):1065-1068. PMID: 34875609
Ogishi M, Yang R, Aytekin C, Langlais D, Bourgey M, Khan T, Ali FA, Rahman M, Delmonte OM, Chrabieh M, Zhang P, Gruber C, Pelham SJ, Spaan AN, Rosain J, Lei WT, Drutman S, Hellmann MD, Callahan MK, Adamow M, Wong P, Wolchok JD, Rao G, Ma CS, Nakajima Y, Yaguchi T, Chamoto K, Williams SC, Emile JF, Rozenberg F, Glickman MS, Rapaport F, Kerner G, Allington G, Tezcan I, Cagdas D, Hosnut FO, Dogu F, Ikinciogullari A, Rao VK, Kainulainen L, Béziat V, Bustamante J, Vilarinho S, Lifton RP, Boisson B, Abel L, Bogunovic D, Marr N, Notarangelo LD, Tangye SG, Honjo T, Gros P, Boisson-Dupuis S, Casanova JL
Nat Med 2021 Sep;27(9):1646-1654. Epub 2021 Jun 28 doi: 10.1038/s41591-021-01388-5. PMID: 34183838Free PMC Article

Diagnosis

Atschekzei F, Traidl S, Carlens J, Schütz K, von Hardenberg S, Elsayed A, Ernst D, Risser L, Thiele T, Graalmann T, Raab J, Baumann U, Witte T, Sogkas G
Front Immunol 2024;15:1400348. Epub 2024 Aug 23 doi: 10.3389/fimmu.2024.1400348. PMID: 39247195Free PMC Article
Song Z, Wu W, Wei W, Xiao W, Lei M, Cai KQ, Huang DW, Jeong S, Zhang JP, Wang H, Kadin ME, Waldmann TA, Staudt LM, Nakagawa M, Yang Y
Blood 2023 Oct 12;142(15):1297-1311. doi: 10.1182/blood.2022019166. PMID: 37339580Free PMC Article
Leiding JW, Vogel TP, Santarlas VGJ, Mhaskar R, Smith MR, Carisey A, Vargas-Hernández A, Silva-Carmona M, Heeg M, Rensing-Ehl A, Neven B, Hadjadj J, Hambleton S, Ronan Leahy T, Meesilpavikai K, Cunningham-Rundles C, Dutmer CM, Sharapova SO, Taskinen M, Chua I, Hague R, Klemann C, Kostyuchenko L, Morio T, Thatayatikom A, Ozen A, Scherbina A, Bauer CS, Flanagan SE, Gambineri E, Giovannini-Chami L, Heimall J, Sullivan KE, Allenspach E, Romberg N, Deane SG, Prince BT, Rose MJ, Bohnsack J, Mousallem T, Jesudas R, Santos Vilela MMD, O'Sullivan M, Pachlopnik Schmid J, Průhová Š, Klocperk A, Rees M, Su H, Bahna S, Baris S, Bartnikas LM, Chang Berger A, Briggs TA, Brothers S, Bundy V, Chan AY, Chandrakasan S, Christiansen M, Cole T, Cook MC, Desai MM, Fischer U, Fulcher DA, Gallo S, Gauthier A, Gennery AR, Gonçalo Marques J, Gottrand F, Grimbacher B, Grunebaum E, Haapaniemi E, Hämäläinen S, Heiskanen K, Heiskanen-Kosma T, Hoffman HM, Gonzalez-Granado LI, Guerrerio AL, Kainulainen L, Kumar A, Lawrence MG, Levin C, Martelius T, Neth O, Olbrich P, Palma A, Patel NC, Pozos T, Preece K, Lugo Reyes SO, Russell MA, Schejter Y, Seroogy C, Sinclair J, Skevofilax E, Suan D, Suez D, Szabolcs P, Velasco H, Warnatz K, Walkovich K, Worth A; STAT3 GOF Working Group members, Seppänen MRJ, Torgerson TR, Sogkas G, Ehl S, Tangye SG, Cooper MA, Milner JD, Forbes Satter LR
J Allergy Clin Immunol 2023 Apr;151(4):1081-1095. Epub 2022 Oct 11 doi: 10.1016/j.jaci.2022.09.002. PMID: 36228738Free PMC Article
López AL, Niemela J, Stoddard J, Paolini MV, Rosenzweig S, Fernández Romero DS
Medicina (B Aires) 2021;81(6):1065-1068. PMID: 34875609
Faletti L, Ehl S, Heeg M
Biomed J 2021 Aug;44(4):412-421. Epub 2021 Mar 20 doi: 10.1016/j.bj.2021.03.003. PMID: 34366294Free PMC Article

Therapy

Atschekzei F, Traidl S, Carlens J, Schütz K, von Hardenberg S, Elsayed A, Ernst D, Risser L, Thiele T, Graalmann T, Raab J, Baumann U, Witte T, Sogkas G
Front Immunol 2024;15:1400348. Epub 2024 Aug 23 doi: 10.3389/fimmu.2024.1400348. PMID: 39247195Free PMC Article
Lan C, Fang G, Qiu C, Li X, Yang F, Yang Y
Clin Exp Hypertens 2024 Dec 31;46(1):2297642. Epub 2023 Dec 26 doi: 10.1080/10641963.2023.2297642. PMID: 38147409
Hadjadj J, Frémond ML, Neven B
Front Immunol 2021;12:717388. Epub 2021 Sep 17 doi: 10.3389/fimmu.2021.717388. PMID: 34603291Free PMC Article
Ogishi M, Yang R, Aytekin C, Langlais D, Bourgey M, Khan T, Ali FA, Rahman M, Delmonte OM, Chrabieh M, Zhang P, Gruber C, Pelham SJ, Spaan AN, Rosain J, Lei WT, Drutman S, Hellmann MD, Callahan MK, Adamow M, Wong P, Wolchok JD, Rao G, Ma CS, Nakajima Y, Yaguchi T, Chamoto K, Williams SC, Emile JF, Rozenberg F, Glickman MS, Rapaport F, Kerner G, Allington G, Tezcan I, Cagdas D, Hosnut FO, Dogu F, Ikinciogullari A, Rao VK, Kainulainen L, Béziat V, Bustamante J, Vilarinho S, Lifton RP, Boisson B, Abel L, Bogunovic D, Marr N, Notarangelo LD, Tangye SG, Honjo T, Gros P, Boisson-Dupuis S, Casanova JL
Nat Med 2021 Sep;27(9):1646-1654. Epub 2021 Jun 28 doi: 10.1038/s41591-021-01388-5. PMID: 34183838Free PMC Article
Turner SD, Inghirami G, Miranda RN, Kadin ME
Am J Pathol 2020 Jan;190(1):2-10. Epub 2019 Oct 11 doi: 10.1016/j.ajpath.2019.09.005. PMID: 31610171Free PMC Article

Prognosis

Marchand T, Lamy T, Loughran TP Jr
Blood 2024 Oct 31;144(18):1910-1923. doi: 10.1182/blood.2023021790. PMID: 38848524
Maccari ME, Wolkewitz M, Schwab C, Lorenzini T, Leiding JW, Aladjdi N, Abolhassani H, Abou-Chahla W, Aiuti A, Azarnoush S, Baris S, Barlogis V, Barzaghi F, Baumann U, Bloomfield M, Bohynikova N, Bodet D, Boutboul D, Bucciol G, Buckland MS, Burns SO, Cancrini C, Cathébras P, Cavazzana M, Cheminant M, Chinello M, Ciznar P, Coulter TI, D'Aveni M, Ekwall O, Eric Z, Eren E, Fasth A, Frange P, Fournier B, Garcia-Prat M, Gardembas M, Geier C, Ghosh S, Goda V, Hammarström L, Hauck F, Heeg M, Heropolitanska-Pliszka E, Hilfanova A, Jolles S, Karakoc-Aydiner E, Kindle GR, Kiykim A, Klemann C, Koletsi P, Koltan S, Kondratenko I, Körholz J, Krüger R, Jeziorski E, Levy R, Le Guenno G, Lefevre G, Lougaris V, Marzollo A, Mahlaoui N, Malphettes M, Meinhardt A, Merlin E, Meyts I, Milota T, Moreira F, Moshous D, Mukhina A, Neth O, Neubert J, Neven B, Nieters A, Nove-Josserand R, Oksenhendler E, Ozen A, Olbrich P, Perlat A, Pac M, Schmid JP, Pacillo L, Parra-Martinez A, Paschenko O, Pellier I, Sefer AP, Plebani A, Plantaz D, Prader S, Raffray L, Ritterbusch H, Riviere JG, Rivalta B, Rusch S, Sakovich I, Savic S, Scheible R, Schleinitz N, Schuetz C, Schulz A, Sediva A, Semeraro M, Sharapova SO, Shcherbina A, Slatter MA, Sogkas G, Soler-Palacin P, Speckmann C, Stephan JL, Suarez F, Tommasini A, Trück J, Uhlmann A, van Aerde KJ, van Montfrans J, von Bernuth H, Warnatz K, Williams T, Worth AJJ, Ip W, Picard C, Catherinot E, Nademi Z, Grimbacher B, Forbes Satter LR, Kracker S, Chandra A, Condliffe AM, Ehl S; European Society for Immunodeficiencies Registry Working Party
J Allergy Clin Immunol 2023 Oct;152(4):984-996.e10. Epub 2023 Jun 28 doi: 10.1016/j.jaci.2023.06.015. PMID: 37390899
Song Z, Wu W, Wei W, Xiao W, Lei M, Cai KQ, Huang DW, Jeong S, Zhang JP, Wang H, Kadin ME, Waldmann TA, Staudt LM, Nakagawa M, Yang Y
Blood 2023 Oct 12;142(15):1297-1311. doi: 10.1182/blood.2022019166. PMID: 37339580Free PMC Article
Turner SD, Inghirami G, Miranda RN, Kadin ME
Am J Pathol 2020 Jan;190(1):2-10. Epub 2019 Oct 11 doi: 10.1016/j.ajpath.2019.09.005. PMID: 31610171Free PMC Article
Suh YW, Horton JC
Ophthalmic Genet 2019 Apr;40(2):165-169. Epub 2019 Apr 3 doi: 10.1080/13816810.2019.1592202. PMID: 30942636Free PMC Article

Clinical prediction guides

Toth KA, Schmitt EG, Kolicheski A, Greenberg ZJ, Levendosky E, Saucier N, Trammel K, Oikonomou V, Lionakis MS, Klechevsky E, Kim BS, Schuettpelz LG, Saligrama N, Cooper MA
J Exp Med 2024 Aug 5;221(8) Epub 2024 Jun 11 doi: 10.1084/jem.20232091. PMID: 38861030Free PMC Article
Marchand T, Lamy T, Loughran TP Jr
Blood 2024 Oct 31;144(18):1910-1923. doi: 10.1182/blood.2023021790. PMID: 38848524
Maccari ME, Wolkewitz M, Schwab C, Lorenzini T, Leiding JW, Aladjdi N, Abolhassani H, Abou-Chahla W, Aiuti A, Azarnoush S, Baris S, Barlogis V, Barzaghi F, Baumann U, Bloomfield M, Bohynikova N, Bodet D, Boutboul D, Bucciol G, Buckland MS, Burns SO, Cancrini C, Cathébras P, Cavazzana M, Cheminant M, Chinello M, Ciznar P, Coulter TI, D'Aveni M, Ekwall O, Eric Z, Eren E, Fasth A, Frange P, Fournier B, Garcia-Prat M, Gardembas M, Geier C, Ghosh S, Goda V, Hammarström L, Hauck F, Heeg M, Heropolitanska-Pliszka E, Hilfanova A, Jolles S, Karakoc-Aydiner E, Kindle GR, Kiykim A, Klemann C, Koletsi P, Koltan S, Kondratenko I, Körholz J, Krüger R, Jeziorski E, Levy R, Le Guenno G, Lefevre G, Lougaris V, Marzollo A, Mahlaoui N, Malphettes M, Meinhardt A, Merlin E, Meyts I, Milota T, Moreira F, Moshous D, Mukhina A, Neth O, Neubert J, Neven B, Nieters A, Nove-Josserand R, Oksenhendler E, Ozen A, Olbrich P, Perlat A, Pac M, Schmid JP, Pacillo L, Parra-Martinez A, Paschenko O, Pellier I, Sefer AP, Plebani A, Plantaz D, Prader S, Raffray L, Ritterbusch H, Riviere JG, Rivalta B, Rusch S, Sakovich I, Savic S, Scheible R, Schleinitz N, Schuetz C, Schulz A, Sediva A, Semeraro M, Sharapova SO, Shcherbina A, Slatter MA, Sogkas G, Soler-Palacin P, Speckmann C, Stephan JL, Suarez F, Tommasini A, Trück J, Uhlmann A, van Aerde KJ, van Montfrans J, von Bernuth H, Warnatz K, Williams T, Worth AJJ, Ip W, Picard C, Catherinot E, Nademi Z, Grimbacher B, Forbes Satter LR, Kracker S, Chandra A, Condliffe AM, Ehl S; European Society for Immunodeficiencies Registry Working Party
J Allergy Clin Immunol 2023 Oct;152(4):984-996.e10. Epub 2023 Jun 28 doi: 10.1016/j.jaci.2023.06.015. PMID: 37390899
Leiding JW, Vogel TP, Santarlas VGJ, Mhaskar R, Smith MR, Carisey A, Vargas-Hernández A, Silva-Carmona M, Heeg M, Rensing-Ehl A, Neven B, Hadjadj J, Hambleton S, Ronan Leahy T, Meesilpavikai K, Cunningham-Rundles C, Dutmer CM, Sharapova SO, Taskinen M, Chua I, Hague R, Klemann C, Kostyuchenko L, Morio T, Thatayatikom A, Ozen A, Scherbina A, Bauer CS, Flanagan SE, Gambineri E, Giovannini-Chami L, Heimall J, Sullivan KE, Allenspach E, Romberg N, Deane SG, Prince BT, Rose MJ, Bohnsack J, Mousallem T, Jesudas R, Santos Vilela MMD, O'Sullivan M, Pachlopnik Schmid J, Průhová Š, Klocperk A, Rees M, Su H, Bahna S, Baris S, Bartnikas LM, Chang Berger A, Briggs TA, Brothers S, Bundy V, Chan AY, Chandrakasan S, Christiansen M, Cole T, Cook MC, Desai MM, Fischer U, Fulcher DA, Gallo S, Gauthier A, Gennery AR, Gonçalo Marques J, Gottrand F, Grimbacher B, Grunebaum E, Haapaniemi E, Hämäläinen S, Heiskanen K, Heiskanen-Kosma T, Hoffman HM, Gonzalez-Granado LI, Guerrerio AL, Kainulainen L, Kumar A, Lawrence MG, Levin C, Martelius T, Neth O, Olbrich P, Palma A, Patel NC, Pozos T, Preece K, Lugo Reyes SO, Russell MA, Schejter Y, Seroogy C, Sinclair J, Skevofilax E, Suan D, Suez D, Szabolcs P, Velasco H, Warnatz K, Walkovich K, Worth A; STAT3 GOF Working Group members, Seppänen MRJ, Torgerson TR, Sogkas G, Ehl S, Tangye SG, Cooper MA, Milner JD, Forbes Satter LR
J Allergy Clin Immunol 2023 Apr;151(4):1081-1095. Epub 2022 Oct 11 doi: 10.1016/j.jaci.2022.09.002. PMID: 36228738Free PMC Article
Lodi L, Faletti LE, Maccari ME, Consonni F, Groß M, Pagnini I, Ricci S, Heeg M, Simonini G, Azzari C, Ehl S
J Allergy Clin Immunol 2022 Nov;150(5):1237-1241.e3. Epub 2022 Jun 21 doi: 10.1016/j.jaci.2022.06.007. PMID: 35750105

Recent systematic reviews

Fabre A, Marchal S, Barlogis V, Mari B, Barbry P, Rohrlich PS, Forbes LR, Vogel TP, Giovannini-Chami L
J Allergy Clin Immunol Pract 2019 Jul-Aug;7(6):1958-1969.e9. Epub 2019 Feb 27 doi: 10.1016/j.jaip.2019.02.018. PMID: 30825606

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