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Metabolite and thymocyte development defects in ADA-SCID mice receiving enzyme replacement therapy

Metabolite and thymocyte development defects in ADA-SCID mice receiving enzyme replacement therapy

https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_doaj_primary_oai_doaj_org_article_f65c7cd9d5074750a94e03ee0d0444ee

Metabolite and thymocyte development defects in ADA-SCID mice receiving enzyme replacement therapy

About this item

Full title

Metabolite and thymocyte development defects in ADA-SCID mice receiving enzyme replacement therapy

Publisher

London: Nature Publishing Group UK

Journal title

Scientific reports, 2021-12, Vol.11 (1), p.23221-17, Article 23221

Language

English

Formats

Publication information

Publisher

London: Nature Publishing Group UK

More information

Scope and Contents

Contents

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 development and function. While untreated ADA-SCID is a f...

Alternative Titles

Full title

Metabolite and thymocyte development defects in ADA-SCID mice receiving enzyme replacement therapy

Identifiers

Primary Identifiers

Record Identifier

TN_cdi_doaj_primary_oai_doaj_org_article_f65c7cd9d5074750a94e03ee0d0444ee

Permalink

https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_doaj_primary_oai_doaj_org_article_f65c7cd9d5074750a94e03ee0d0444ee

Other Identifiers

ISSN

2045-2322

E-ISSN

2045-2322

DOI

10.1038/s41598-021-02572-w

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