|ZFIN ID: ZDB-PUB-100719-44|
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Developing T lymphocytes are uniquely sensitive to a lack of topoisomerase III alpha
Mönnich, M., Hess, I., Wiest, W., Bachrati, C., Hickson, I.D., Schorpp, M., and Boehm, T.
|Source:||European journal of immunology 40(9): 2379-2384 (Journal)|
|Registered Authors:||Boehm, Tom, Hess, Isabell, Monnich, Maren, Schorpp, Michael, Wiest, Waltraud|
|Keywords:||thymopoiesis, topoisomerase IIIa, mutation, zebrafish|
|PubMed:||20623552 Full text @ Eur. J. Immunol.|
Mönnich, M., Hess, I., Wiest, W., Bachrati, C., Hickson, I.D., Schorpp, M., and Boehm, T. (2010) Developing T lymphocytes are uniquely sensitive to a lack of topoisomerase III alpha. European journal of immunology. 40(9):2379-2384.
ABSTRACTAll organisms possess at least one typeDNA topoisomerase. These topoisomerases function as part of a DNA structure-specific "dissolvasome", also known as the RTR complex, which has critical functions in faithful DNA replication, recombination and chromosome segregation. In humans, the heteromeric RTR complex consists of RMI1, RMI2, the Bloom's syndrome gene product (BLM), and topoisomerase 3A (TOP3A) proteins. Here, we describe the identification and characterization of two deleterious mutations in the zebrafish top3agene that reveal an unexpected tissue-specific requirement of top3a function in developing thymocytes. Deficiency in top3a activates a p53-dependent check-point but does not affect VDJ recombination. Our results suggest that TOP3A could be a candidate gene involved in human primary immunodeficiency syndromes.