1ols

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1ols, resolution 1.85Å

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ROLES OF HIS291-ALPHA AND HIS146-BETA' IN THE REDUCTIVE ACYLATION REACTION CATALYZED BY HUMAN BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE

Disease

Known diseases associated with this structure: Maple syrup urine disease, type Ia OMIM:[608348], Maple syrup urine disease, type Ib OMIM:[248611]

About this Structure

1OLS is a Protein complex structure of sequences from Homo sapiens with , , and as ligands. Active as 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring), with EC number 1.2.4.4 Known structural/functional Site: . Full crystallographic information is available from OCA.

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