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Mouse modeling and structural analysis of the p.G307S mutation in human cystathionine β-synthase (CBS) reveal effects on CBS activity but not stability

Mutations in the cystathionine β-synthase (CBS) gene are the cause of classical homocystinuria, the most common inborn error in sulfur metabolism. The p.G307S mutation is the most frequent cause of CBS deficiency in Ireland, which has the highest prevalence of CBS deficiency in Europe. Individuals h...

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Bibliografiske detaljer
Udgivet i:J Biol Chem
Main Authors: Gupta, Sapna, Kelow, Simon, Wang, Liqun, Andrake, Mark D., Dunbrack, Roland L., Kruger, Warren D.
Format: Artigo
Sprog:Inglês
Udgivet: American Society for Biochemistry and Molecular Biology 2018
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6130948/
https://ncbi.nlm.nih.gov/pubmed/30030379
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.RA118.002164
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