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1u5b

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{{STRUCTURE_1u5b| PDB=1u5b | SCENE= }}
{{STRUCTURE_1u5b| PDB=1u5b | SCENE= }}
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'''Crystal structure of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase'''
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===Crystal structure of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase===
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==Overview==
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The human mitochondrial branched-chain alpha-ketoacid dehydrogenase complex (BCKDC) is a 4 MDa macromolecular machine comprising three catalytic components (E1b, E2b, and E3), a kinase, and a phosphatase. The BCKDC overall activity is tightly regulated by phosphorylation in response to hormonal and dietary stimuli. We report that phosphorylation of Ser292-alpha in the E1b active site channel results in an order-to-disorder transition of the conserved phosphorylation loop carrying the phosphoryl serine. The conformational change is triggered by steric clashes of the phosphoryl group with invariant His291-alpha that serves as an indispensable anchor for the phosphorylation loop through bound thiamin diphosphate. Phosphorylation of Ser292-alpha does not severely impede the E1b-dependent decarboxylation of alpha-ketoacids. However, the disordered loop conformation prevents phosphorylated E1b from binding the E2b lipoyl-bearing domain, which effectively shuts off the E1b-catalyzed reductive acylation reaction and therefore completely inactivates BCKDC. This mechanism provides a paradigm for regulation of mitochondrial alpha-ketoacid dehydrogenase complexes by phosphorylation.
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{{ABSTRACT_PUBMED_15576032}}
==Disease==
==Disease==
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[[Category: Phosphorylation]]
[[Category: Phosphorylation]]
[[Category: Thiamin diphosphate]]
[[Category: Thiamin diphosphate]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 10:46:22 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 29 14:30:56 2008''

Revision as of 11:30, 29 July 2008

Image:1u5b.png

Template:STRUCTURE 1u5b

Contents

Crystal structure of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase

Template:ABSTRACT PUBMED 15576032

Disease

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

About this Structure

1U5B is a Protein complex structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Molecular mechanism for regulation of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase complex by phosphorylation., Wynn RM, Kato M, Machius M, Chuang JL, Li J, Tomchick DR, Chuang DT, Structure. 2004 Dec;12(12):2185-96. PMID:15576032

Page seeded by OCA on Tue Jul 29 14:30:56 2008

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