1x7y

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[[Image:1x7y.jpg|left|200px]]
[[Image:1x7y.jpg|left|200px]]
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{{Structure
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<!--
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|PDB= 1x7y |SIZE=350|CAPTION= <scene name='initialview01'>1x7y</scene>, resolution 1.57&Aring;
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The line below this paragraph, containing "STRUCTURE_1x7y", creates the "Structure Box" on the page.
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|SITE=
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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|LIGAND= <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=K:POTASSIUM+ION'>K</scene>, <scene name='pdbligand=MN:MANGANESE+(II)+ION'>MN</scene>, <scene name='pdbligand=TDP:THIAMIN+DIPHOSPHATE'>TDP</scene>
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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|ACTIVITY= <span class='plainlinks'>[http://en.wikipedia.org/wiki/3-methyl-2-oxobutanoate_dehydrogenase_(2-methylpropanoyl-transferring) 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.2.4.4 1.2.4.4] </span>
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or leave the SCENE parameter empty for the default display.
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|GENE= BCKDHA ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens]), BCKDHB ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
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-->
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|DOMAIN=
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{{STRUCTURE_1x7y| PDB=1x7y | SCENE= }}
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|RELATEDENTRY=[[1u5b|1U5B]]
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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1x7y FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1x7y OCA], [http://www.ebi.ac.uk/pdbsum/1x7y PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1x7y RCSB]</span>
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}}
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'''Crystal structure of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase'''
'''Crystal structure of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase'''
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==Reference==
==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:[http://www.ncbi.nlm.nih.gov/pubmed/15576032 15576032]
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:[http://www.ncbi.nlm.nih.gov/pubmed/15576032 15576032]
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[[Category: 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)]]
 
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Protein complex]]
[[Category: Protein complex]]
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[[Category: Tomchick, D R.]]
[[Category: Tomchick, D R.]]
[[Category: Wynn, R M.]]
[[Category: Wynn, R M.]]
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[[Category: acylation]]
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[[Category: Acylation]]
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[[Category: branched-chain]]
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[[Category: Branched-chain]]
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[[Category: flavoprotein]]
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[[Category: Flavoprotein]]
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[[Category: multi-enzyme complex]]
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[[Category: Multi-enzyme complex]]
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[[Category: oxidative decarboxylation maple syrup urine disease]]
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[[Category: Oxidative decarboxylation maple syrup urine disease]]
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[[Category: oxidoreductase,ketoacid dehydrogenase]]
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[[Category: Oxidoreductase,ketoacid dehydrogenase]]
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[[Category: phosphorylation]]
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[[Category: Phosphorylation]]
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[[Category: thiamin diphosphate]]
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[[Category: Thiamin diphosphate]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 14:41:02 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Mar 31 00:45:54 2008''
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Revision as of 11:41, 3 May 2008

Image:1x7y.jpg

Template:STRUCTURE 1x7y

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


Contents

Overview

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.

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

1X7Y 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 Sat May 3 14:41:02 2008

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OCA

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