2j9f
From Proteopedia
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HUMAN BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE-DECARBOXYLASE E1B
Disease
Known disease associated with this structure: Maple syrup urine disease, type Ia OMIM:[608348]
About this Structure
2J9F is a Protein complex structure of sequences from Homo sapiens with MN, K, THV and GOL as ligands. Active as 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring), with EC number 1.2.4.4 Structure known Active Site: AC1. Full crystallographic information is available from OCA.
Page seeded by OCA on Mon Nov 12 22:53:21 2007
Categories: 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) | Homo sapiens | Protein complex | Chuang, D.T. | Chuang, J.L. | Jun, L. | Machius, M. | Wynn, R.M. | GOL | K | MN | THV | Branched-chain | Disease mutation | Flavoprotein | Ketoacid dehydrogenase | Maple syrup urine disease | Metal-binding | Mitochondrion | Multi-enzyme complex | Oxidoreductase | Phosphorylation | Potassium | Thiamine pyrophosphate | Transit peptide
