BBLN, a protein with largely unknown function, was found to be upregulated in damaged hearts of children with tetralogy of Fallot, one of the most frequent congenital heart defects. Transgenic mice and in vitro studies showed that elevated BBLN levels triggered heart damage by activation of the protein CAMK2D.
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References
Wilson, R., Ross, O. & Griksaitis, M. J. Tetralogy of Fallot. BJA Educ. 19, 362–369 (2019). This review describes the prevalence, major cardiac anomalies and clinical features of TOF.
Ishikita, A., Friedberg, M. K. & Wald, R. M. Right ventricular fibrosis after tetralogy of Fallot repair: can pulmonary valve replacement make a difference? JACC Basic Transl. Sci. 8, 316–318 (2023). This editorial highlights the major long-term complications of repaired TOF and raises open questions for future research.
Willeford, A. et al. CaMKIIδ-mediated inflammatory gene expression and inflammasome activation in cardiomyocytes initiate inflammation and induce fibrosis. JCI Insight. 3, e97054 (2018). This article linked CAMK2D-triggered pathways to inflammation and cardiac fibrosis, which are also induced by BBLN-enhanced CAMK2D activation.
Nauffal, V. et al. Genetics of myocardial interstitial fibrosis in the human heart and association with disease. Nat. Genet. 55, 777–786 (2023). This article analyzed data from over 40,000 study participants and identified CAMK2D as part of a biological pathway involved in myocardial fibrosis and adverse prognosis in patients with cardiovascular disease.
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This is a summary of: Abd Alla, J. et al. BBLN triggers CAMK2D pathology in mice under cardiac pressure overload and potentially in unrepaired hearts with tetralogy of Fallot. Nat. Cardiovasc. Res. https://doi.org/10.1038/s44161-023-00351-6 (2023).
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Elevated BBLN levels as a cause of heart defects in tetralogy of Fallot. Nat Cardiovasc Res 2, 970–971 (2023). https://doi.org/10.1038/s44161-023-00360-5
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DOI: https://doi.org/10.1038/s44161-023-00360-5
