Abstract
Mechanisms underlying the pathophysiology of minimal-change nephrotic syndrome (MCNS), the most frequent glomerular disease in children, remain elusive, but recent findings argue for a T cell dysfunction. Starting from a differential cDNA library from T cells of a patient under relapse and remission, we identified 16 transcripts specific for MCNS. All of these transcripts that were selectively up-regulated during the relapse phase of the disease were generated by alternative splicing of known genes. This abnormal RNA expression was associated with a down-regulation of serin-rich protein 75 and serin-rich protein 40, two proteins involved in mRNA splicing. Taken together, these data suggest that T cell dysfunction in MCNS is associated with abnormal mRNA splicing.
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Abbreviations
- MCNS:
-
minimal-change nephrotic syndrome
- PBMC:
-
peripheral blood mononuclear cell
- SR:
-
serin-rich
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Acknowledgements
We are indebted to Drs. P Remy, V. Baudouin, P. Niaudet, and M. Broyer for providing blood samples and clinical information as well as for support and advice.
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This work was supported in part by the Association pour l'Utilisation du Rein Artificiel (AURA, Paris) and an apex grant from the INSERM.V.A. and A.V. contributed equally to this work.
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Grimbert, P., Audard, V., Valanciute, A. et al. Abnormal RNA Processing and Altered Expression of Serin-Rich Proteins in Minimal-Change Nephrotic Syndrome. Pediatr Res 57, 133–137 (2005). https://doi.org/10.1203/01.PDR.0000148013.53429.5B
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DOI: https://doi.org/10.1203/01.PDR.0000148013.53429.5B
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