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Summary Structural Experimental Function Sequence Neighbor Download Link


<Asymmetric unit>

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PDB ID1dtw  sequence information (FASTA format)   
DescriptorHUMAN BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE (E.C.1.2.4.4)
TitleHUMAN BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE
Functional KeywordsThDP-binding fold, BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE, OXIDOREDUCTASE
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 weight84000.9 (the details in Structural Details Page)
AuthorsAEvarsson, A. , Chuang, J.L. , Wynn, R.M. , Turley, S. , Chuang, D.T. , Hol, W.G.J. (deposition date : 2000-01-13, release date : 2000-03-27)
Primary citationAEvarsson, A. , Chuang, J.L. , Wynn, R.M. , Turley, S. , Chuang, D.T. , Hol, W.G.
Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.
Structure, 8:277 - 291, 2000.(PubMed : 10745006)  (DOI: 10.1016/S0969-2126(00)00105-2)
Experimental methodX-RAY DIFFRACTION ( 2.70[Å] )
Other Database Information
Yorodumi , CATH , CE , FSSP , SCOP , VAST , UniProt ( P12694, P21953 ) , eF-site , KEGG ( EC 1.2.4.4 ) , PISA