The TRIM37 variants in Mulibrey nanism patients paralyze follicular helper T cell differentiation

  1. Gu, Wangpeng
  2. Zhang, Jia
  3. Li, Qing ORCID logo
  4. Zhang, Yaguang ORCID logo
  5. Lin, Xuan
  6. Wu, Bingbing
  7. Yin, Qi
  8. Sun, Jinqiao
  9. Lu, Yulan ORCID logo
  10. Sun, Xiaoyu
  11. Jia, Caiwei
  12. Li, Chuanyin
  13. Zhang, Yu
  14. Wang, Meng
  15. Yin, Xidi
  16. Wang, Su
  17. Xu, Jiefang
  18. Wang, Ran
  19. Zhu, Songling
  20. Cheng, Shipeng
  21. Chen, Shuangfeng
  22. Liu, Lian
  23. Zhu, Lin
  24. Yan, Chenghua
  25. Yi, Chunyan
  26. Li, Xuezhen
  27. Lian, Qiaoshi
  28. Lin, Guomei
  29. Ling, Zhiyang
  30. Ma, Liyan
  31. Zhou, Min
  32. Xiao, Kuanlin
  33. Wei, Haiming
  34. Hu, Ronggui
  35. Zhou, Wenhao ORCID logo
  36. Ye, Lilin ORCID logo
  37. 王, 海坤 ORCID logo
  38. 李, 劲松 ORCID logo
  39. Sun, Bing

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WeightNameValue
1000 Titel
  • The TRIM37 variants in Mulibrey nanism patients paralyze follicular helper T cell differentiation
1000 Autor/in
  1. Gu, Wangpeng |
  2. Zhang, Jia |
  3. Li, Qing |
  4. Zhang, Yaguang |
  5. Lin, Xuan |
  6. Wu, Bingbing |
  7. Yin, Qi |
  8. Sun, Jinqiao |
  9. Lu, Yulan |
  10. Sun, Xiaoyu |
  11. Jia, Caiwei |
  12. Li, Chuanyin |
  13. Zhang, Yu |
  14. Wang, Meng |
  15. Yin, Xidi |
  16. Wang, Su |
  17. Xu, Jiefang |
  18. Wang, Ran |
  19. Zhu, Songling |
  20. Cheng, Shipeng |
  21. Chen, Shuangfeng |
  22. Liu, Lian |
  23. Zhu, Lin |
  24. Yan, Chenghua |
  25. Yi, Chunyan |
  26. Li, Xuezhen |
  27. Lian, Qiaoshi |
  28. Lin, Guomei |
  29. Ling, Zhiyang |
  30. Ma, Liyan |
  31. Zhou, Min |
  32. Xiao, Kuanlin |
  33. Wei, Haiming |
  34. Hu, Ronggui |
  35. Zhou, Wenhao |
  36. Ye, Lilin |
  37. 王, 海坤 |
  38. 李, 劲松 |
  39. Sun, Bing |
1000 Erscheinungsjahr 2023
1000 Publikationstyp
  1. Artikel |
1000 Online veröffentlicht
  • 2023-08-01
1000 Erschienen in
1000 Quellenangabe
  • 9(1):82
1000 Copyrightjahr
  • 2023
1000 Lizenz
1000 Verlagsversion
  • https://doi.org/10.1038/s41421-023-00561-z |
  • https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10394018/ |
1000 Ergänzendes Material
  • https://www.nature.com/articles/s41421-023-00561-z#Sec28 |
1000 Publikationsstatus
1000 Begutachtungsstatus
1000 Sprache der Publikation
1000 Abstract/Summary
  • The Mulibrey (Muscle–liver–brain–eye) nanism caused by loss-of-function variants in TRIM37 gene is an autosomal recessive disorder characterized by severe growth failure and constrictive pericarditis. These patients also suffer from severe respiratory infections, co-incident with an increased mortality rate. Here, we revealed that TRIM37 variants were associated with recurrent infection. Trim37 FINmajor (a representative variant of Mulibrey nanism patients) and Trim37 knockout mice were susceptible to influenza virus infection. These mice showed defects in follicular helper T (TFH) cell development and antibody production. The effects of Trim37 on TFH cell differentiation relied on its E3 ligase activity catalyzing the K27/29-linked polyubiquitination of Bcl6 and its MATH domainmediated interactions with Bcl6, thereby protecting Bcl6 from proteasome-mediated degradation. Collectively, these findings highlight the importance of the Trim37-Bcl6 axis in controlling the development of TFH cells and the production of high-affinity antibodies, and further unveil the immunologic mechanism underlying recurrent respiratory infection in Mulibrey nanism.
1000 Sacherschließung
lokal Immunology
lokal Mechanisms of disease
lokal Ubiquitylation
1000 Fächerklassifikation (DDC)
1000 Liste der Beteiligten
  1. https://frl.publisso.de/adhoc/uri/R3UsIFdhbmdwZW5n|https://frl.publisso.de/adhoc/uri/WmhhbmcsIEppYQ==|https://orcid.org/0000-0002-6392-9699|https://orcid.org/0000-0002-2417-6516|https://frl.publisso.de/adhoc/uri/TGluLCBYdWFu|https://frl.publisso.de/adhoc/uri/V3UsIEJpbmdiaW5n|https://frl.publisso.de/adhoc/uri/WWluLCBRaQ==|https://frl.publisso.de/adhoc/uri/U3VuLCBKaW5xaWFv|https://orcid.org/0000-0003-4941-5736|https://frl.publisso.de/adhoc/uri/U3VuLCBYaWFveXU=|https://frl.publisso.de/adhoc/uri/SmlhLCBDYWl3ZWk=|https://frl.publisso.de/adhoc/uri/TGksIENodWFueWlu|https://frl.publisso.de/adhoc/uri/WmhhbmcsIFl1|https://frl.publisso.de/adhoc/uri/V2FuZywgTWVuZyA=|https://frl.publisso.de/adhoc/uri/WWluLCBYaWRp|https://frl.publisso.de/adhoc/uri/V2FuZywgU3U=|https://frl.publisso.de/adhoc/uri/WHUsIEppZWZhbmc=|https://frl.publisso.de/adhoc/uri/V2FuZywgUmFuIA==|https://frl.publisso.de/adhoc/uri/Wmh1LCBTb25nbGluZyA=|https://frl.publisso.de/adhoc/uri/Q2hlbmcsIFNoaXBlbmcg|https://frl.publisso.de/adhoc/uri/Q2hlbiwgU2h1YW5nZmVuZyA=|https://frl.publisso.de/adhoc/uri/IExpdSwgTGlhbiA=|https://frl.publisso.de/adhoc/uri/Wmh1LCBMaW4g|https://frl.publisso.de/adhoc/uri/WWFuLCBDaGVuZ2h1YSA=|https://frl.publisso.de/adhoc/uri/WWksIENodW55YW4g|https://frl.publisso.de/adhoc/uri/TGksIFh1ZXpoZW4=|https://frl.publisso.de/adhoc/uri/TGlhbiwgUWlhb3NoaQ==|https://frl.publisso.de/adhoc/uri/TGluLCBHdW9tZWk=|https://frl.publisso.de/adhoc/uri/TGluZywgWmhpeWFuZw==|https://frl.publisso.de/adhoc/uri/TWEsIExpeWFu|https://frl.publisso.de/adhoc/uri/WmhvdSwgTWluIA==|https://frl.publisso.de/adhoc/uri/WGlhbywgS3VhbmxpbiA=|https://frl.publisso.de/adhoc/uri/V2VpLCBIYWltaW5nIA==|https://frl.publisso.de/adhoc/uri/SHUsIFJvbmdndWk=|https://orcid.org/0000-0001-8956-7238|https://orcid.org/0000-0003-0778-3311|https://orcid.org/0000-0002-4714-4672|https://orcid.org/0000-0003-3456-662X|https://frl.publisso.de/adhoc/uri/U3VuLCBCaW5nIA==
1000 Label
1000 Förderer
  1. Ministry of Science and Technology of China |
  2. Shanghai Science and Technology Innovation Action |
  3. Strategic Priority Research Program of the Chinese Academy of Sciences |
  4. National Natural Science Foundation of China |
  5. National Key Research and Development Program of China |
  6. Shanghai Municipal Science and Technology Major Project |
  7. Genome Tagging Project (GTP), SIBCB |
1000 Fördernummer
  1. 2018YFA0507402
  2. 21JC1405800;20S11901800
  3. XDB29030103
  4. 3210060158
  5. 2021YFA1301402;2022YFC2703603
  6. ZD2021CY001
  7. -
1000 Förderprogramm
  1. -
  2. -
  3. -
  4. -
  5. -
  6. -
  7. -
1000 Dateien
1000 Förderung
  1. 1000 joinedFunding-child
    1000 Förderer Ministry of Science and Technology of China |
    1000 Förderprogramm -
    1000 Fördernummer 2018YFA0507402
  2. 1000 joinedFunding-child
    1000 Förderer Shanghai Science and Technology Innovation Action |
    1000 Förderprogramm -
    1000 Fördernummer 21JC1405800;20S11901800
  3. 1000 joinedFunding-child
    1000 Förderer Strategic Priority Research Program of the Chinese Academy of Sciences |
    1000 Förderprogramm -
    1000 Fördernummer XDB29030103
  4. 1000 joinedFunding-child
    1000 Förderer National Natural Science Foundation of China |
    1000 Förderprogramm -
    1000 Fördernummer 3210060158
  5. 1000 joinedFunding-child
    1000 Förderer National Key Research and Development Program of China |
    1000 Förderprogramm -
    1000 Fördernummer 2021YFA1301402;2022YFC2703603
  6. 1000 joinedFunding-child
    1000 Förderer Shanghai Municipal Science and Technology Major Project |
    1000 Förderprogramm -
    1000 Fördernummer ZD2021CY001
  7. 1000 joinedFunding-child
    1000 Förderer Genome Tagging Project (GTP), SIBCB |
    1000 Förderprogramm -
    1000 Fördernummer -
1000 Objektart article
1000 Beschrieben durch
1000 @id frl:6462214.rdf
1000 Erstellt am 2023-10-27T10:28:26.848+0200
1000 Erstellt von 284
1000 beschreibt frl:6462214
1000 Bearbeitet von 317
1000 Zuletzt bearbeitet 2023-10-31T07:44:51.060+0100
1000 Objekt bearb. Tue Oct 31 07:44:37 CET 2023
1000 Vgl. frl:6462214
1000 Oai Id
  1. oai:frl.publisso.de:frl:6462214 |
1000 Sichtbarkeit Metadaten public
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