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Female hyper IgM syndrome type 1 with a chromosomal translocation disrupting CD40LG.
Imai K, Shimadzu M, Kubota T, Morio T, Matsunaga T, Park YD, Yoshioka A, Nonoyama S. Imai K, et al. Biochim Biophys Acta. 2006 Mar;1762(3):335-40. doi: 10.1016/j.bbadis.2005.10.003. Epub 2005 Oct 28. Biochim Biophys Acta. 2006. PMID: 16311023 Free article.
Here, we report the second case of a female HIGM1 with the defect of CD40 ligand (CD40L) expression and of soluble serum CD40L. Clinical course and HIGM phenotype was indistinguishable from that of male HIGM1 including severe neutropenia. ...
Here, we report the second case of a female HIGM1 with the defect of CD40 ligand (CD40L) expression and of soluble serum CD40L. Clinical …
Integrin Binding to the Trimeric Interface of CD40L Plays a Critical Role in CD40/CD40L Signaling.
Takada YK, Yu J, Shimoda M, Takada Y. Takada YK, et al. J Immunol. 2019 Sep 1;203(5):1383-1391. doi: 10.4049/jimmunol.1801630. Epub 2019 Jul 22. J Immunol. 2019. PMID: 31331973
In this article, we describe that the integrin-binding site of human CD40L is predicted to be located in the trimeric interface by docking simulation. Mutations in the predicted integrin-binding site markedly reduced the binding of alpha5beta1 to CD40L. ...
In this article, we describe that the integrin-binding site of human CD40L is predicted to be located in the trimeric interface by do …
Soluble CD40L activates soluble and cell-surface integrin αvβ3, α5β1, and α4β1 by binding to the allosteric ligand-binding site (site 2).
Takada YK, Shimoda M, Maverakis E, Felding BH, Cheng RH, Takada Y. Takada YK, et al. J Biol Chem. 2021 Jan-Jun;296:100399. doi: 10.1016/j.jbc.2021.100399. Epub 2021 Feb 9. J Biol Chem. 2021. PMID: 33571526 Free PMC article.
To learn more about the mechanism of binding, we determined that sCD40L bound to a cyclic peptide from site 2. Docking simulations predicted that the residues of CD40L that bind to site 2 are located outside of the CD40L trimer interface, at a site where four HIGM1 (hyper- …
To learn more about the mechanism of binding, we determined that sCD40L bound to a cyclic peptide from site 2. Docking simulations predic …
ARDS as presenting symptom in an infant with CD40L deficiency (Hyper-IgM syndrome Type 1).
Fremerey C, Wiebe B, Feyen O, Lenski C, Pohlmann U, Ehlen M, Schofer O, Meindl A, Niehues T, Bartmann P. Fremerey C, et al. Klin Padiatr. 2009 Sep;221(5):302-4. doi: 10.1055/s-0029-1192041. Epub 2009 Aug 25. Klin Padiatr. 2009. PMID: 19707993
The case underlines the importance of an extended diagnostic work-up in an uncommonly severe course of respiratory infection in early infancy....
The case underlines the importance of an extended diagnostic work-up in an uncommonly severe course of respiratory infection in early …
Cryptosporidium infection in patients with primary immunodeficiencies.
Wolska-Kusnierz B, Bajer A, Caccio S, Heropolitanska-Pliszka E, Bernatowska E, Socha P, van Dongen J, Bednarska M, Paziewska A, Sinski E. Wolska-Kusnierz B, et al. J Pediatr Gastroenterol Nutr. 2007 Oct;45(4):458-64. doi: 10.1097/MPG.0b013e318054b09b. J Pediatr Gastroenterol Nutr. 2007. PMID: 18030213