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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...

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Detalles Bibliográficos
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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2021
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