Characterization and structure of the human lysine-2-oxoglutarate reductase domain, a novel therapeutic target for treatment of glutaric aciduria type 1

Characterization and structure of the human lysine-2-oxoglutarate reductase domain, a novel therapeutic target for treatment of glutaric aciduria type 1

In humans, a single enzyme 2-aminoadipic semialdehyde synthase (AASS) catalyses the initial two critical reactions in the lysine degradation pathway. This enzyme evolved to be a bifunctional enzyme with both lysine-2-oxoglutarate reductase (LOR) and saccharopine dehydrogenase domains (SDH). Moreover...

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Bibliographic Details
Main Authors: João Leandro, Susmita Khamrui, Chalada Suebsuwong, Peng-Jen Chen, Cody Secor, Tetyana Dodatko, Chunli Yu, Roberto Sanchez, Robert J. DeVita, Sander M. Houten, Michael B. Lazarus
Format: Article
Language:English
Published: The Royal Society 2022-09-01
Series:Open Biology
Subjects:
crystal structure
glutaric aciduria
inborn errors of metabolism
lysine metabolism
enzymology
assay development
Online Access:https://royalsocietypublishing.org/doi/10.1098/rsob.220179

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https://royalsocietypublishing.org/doi/10.1098/rsob.220179

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