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6I4P

Crystal structure of the disease-causing G194C mutant of the human dihydrolipoamide dehydrogenase

Entity
Entity IDChain IDDescriptionTypeChain lengthFormula weightNumber of moleculesDB Name (Accession)Biological sourceDescriptive keywords
1A, B
(A, B)
Dihydrolipoyl dehydrogenase, mitochondrialpolymer49652683.32UniProt (P09622)
Pfam (PF07992)
Pfam (PF02852)
Homo sapiens (Human)Dihydrolipoamide dehydrogenase,Glycine cleavage system L protein
2C, J
(A, B)
FLAVIN-ADENINE DINUCLEOTIDEnon-polymer785.52Chemie (FAD)
3D, E, F, G, H...
(A, B)
SULFATE IONnon-polymer96.19Chemie (SO4)
4I, O
(A, B)
2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOLnon-polymer209.22Chemie (BTB)
5P, Q
(A, B)
waterwater18.0587Chemie (HOH)
Sequence modifications
A, B: 1 - 474 (UniProt: P09622)
PDBExternal DatabaseDetails
Met -21-initiating methionine
Ala -20-expression tag
Ser -19-expression tag
Trp -18-expression tag
Ser -17-expression tag
His -16-expression tag
Pro -15-expression tag
Gln -14-expression tag
Phe -13-expression tag
Glu -12-expression tag
Lys -11-expression tag
Gly -10-expression tag
Ala -9-expression tag
Leu -8-expression tag
Glu -7-expression tag
Val -6-expression tag
Leu -5-expression tag
Phe -4-expression tag
Gln -3-expression tag
Gly -2-expression tag
Pro -1-expression tag
Gly 0-expression tag
Cys 194Gly 229engineered mutation
Sequence viewer
Contents of the asymmetric unit
PolymersNumber of chains2
Total formula weight105366.5
Non-Polymers*Number of molecules13
Total formula weight2854.1
All*Total formula weight108220.7
*Water molecules are not included.

246905

PDB entries from 2025-12-31

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