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Summary Structural Experimental Function Sequence Neighbor Download Link


<Asymmetric unit>

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( jV3 / Jmol ) *1
PDB ID2bfd  sequence information (FASTA format)   
RELATED PDB ID1dtw, 1ols, 1olu, 1olx, 1u5b, 1v11, 1v16, 1v1m, 1v1r, 1wci, 1x7w, 1x7x, 1x7y, 1x7z, 1x80, 2beu, 2bev, 2bew, 2bfb, 2bfc, 2bfe, 2bff
Descriptor2-OXOISOVALERATE DEHYDROGENASE ALPHA SUBUNIT (E.C.1.2.4.4), 2-OXOISOVALERATE DEHYDROGENASE BETA SUBUNIT (E.C.1.2.4.4)
TitleREACTIVITY MODULATION OF HUMAN BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE BY AN INTERNAL MOLECULAR SWITCH
Functional KeywordsOXIDOREDUCTASE, KETOACID DEHYDROGENASE, BRANCHED-CHAIN, MULTI- ENZYME COMPLEX, ACYLATION, OXIDATIVE DECARBOXYLATION, MAPLE SYRUP URINE DISEASE, THIAMINE DIPHOSPHATE, PHOSPHORYLATION, CONFORMATIONAL SWITCH, REACTIVITY
Biological sourceHOMO SAPIENS (HUMAN)
Cellular location [UNP - P12694] Mitochondrion matrix
Organ source [UNP - P12694] Bone marrow, and Lung
[UNP - P12694] Liver
[UNP - P12694] Epithelium
[UNP - P12694] Platelet
[UNP - P21953] Placenta
[UNP - P21953] Eye
Total number of polymer chains2
Total molecular weight84102.1 (the details in Structural Details Page)
AuthorsMachius, M. , Wynn, R.M. , Chuang, J.L. , Tomchick, D.R. , Brautigam, C.A. , Chuang, D.T. (deposition date : 2004-12-06, release date : 2006-02-16)
Primary citationMachius, M. , Wynn, R.M. , Chuang, J.L. , Li, J. , Kluger, R. , Yu, D. , Tomchick, D.R. , Brautigam, C.A. , Chuang, D.T.
A Versatile Conformational Switch Regulates Reactivity in Human Branched-Chain Alpha-Ketoacid Dehydrogenase.
Structure, 14:287 - , 2006.(PubMed : 16472748)  (DOI: 10.1016/J.STR.2005.10.009)
Experimental methodX-RAY DIFFRACTION ( 1.39[Å] )
Other Database Information
Yorodumi , CATH , CE , FSSP , SCOP , VAST , UniProt ( P12694, P21953 ) , eF-site , KEGG ( EC 1.2.4.4 ) , PISA