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Glutamate dehydrogenase: Structure of a hyperinsulinism mutant, corrections to the atomic model, and insights into a regulatory site

Mammalian glutamate dehydrogenase (GDH) has complex allosteric regulation and the loss of GTP inhibition causes the hyperinsulinism/hyperammonemia syndrome (HHS) where insulin is hypersecreted upon consumption of protein. The archetypical HHS lesion is H454Y and lies in the GTP binding pocket. To be...

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Detalhes bibliográficos
Publicado no:Proteins
Main Authors: Nassar, Omneya M., Li, Changhong, Stanley, Charles A., Pettitt, B. Montgomery, Smith, Thomas J.
Formato: Artigo
Idioma:Inglês
Publicado em: 2018
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6469361/
https://ncbi.nlm.nih.gov/pubmed/30367518
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/prot.25620
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