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Metabolite and thymocyte development defects in ADA-SCID mice receiving enzyme replacement therapy
Deficiency of adenosine deaminase (ADA, EC3.5.4.4), a housekeeping enzyme intrinsic to the purine salvage pathway, leads to severe combined immunodeficiency (SCID) both in humans and mice. Lack of ADA results in the intracellular accumulation of toxic metabolites which have effects on T cell develop...
Autores principales: | Moretti, Federico A., Giardino, Giuliana, Attenborough, Teresa C. H., Gkazi, Athina Soragia, Margetts, Ben K., la Marca, Giancarlo, Fairbanks, Lynette, Crompton, Tessa, Gaspar, H. Bobby |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8636570/ https://www.ncbi.nlm.nih.gov/pubmed/34853379 http://dx.doi.org/10.1038/s41598-021-02572-w |
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