6I4P
Crystal structure of the disease-causing G194C mutant of the human dihydrolipoamide dehydrogenase
Entity
| Entity ID | Chain ID | Description | Type | Chain length | Formula weight | Number of molecules | DB Name (Accession) | Biological source | Descriptive keywords |
| 1 | A, B (A, B) | Dihydrolipoyl dehydrogenase, mitochondrial | polymer | 496 | 52683.3 | 2 | UniProt (P09622) Pfam (PF07992) Pfam (PF02852) | Homo sapiens (Human) | Dihydrolipoamide dehydrogenase,Glycine cleavage system L protein |
| 2 | C, J (A, B) | FLAVIN-ADENINE DINUCLEOTIDE | non-polymer | 785.5 | 2 | Chemie (FAD) | |||
| 3 | D, E, F, G, H... (A, B) | SULFATE ION | non-polymer | 96.1 | 9 | Chemie (SO4) | |||
| 4 | I, O (A, B) | 2-[BIS-(2-HYDROXY-ETHYL)-AMINO]-2-HYDROXYMETHYL-PROPANE-1,3-DIOL | non-polymer | 209.2 | 2 | Chemie (BTB) | |||
| 5 | P, Q (A, B) | water | water | 18.0 | 587 | Chemie (HOH) |
Sequence modifications
A, B: 1 - 474 (UniProt: P09622)
| PDB | External Database | Details |
|---|---|---|
| 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 194 | Gly 229 | engineered mutation |
Sequence viewer
Contents of the asymmetric unit
| Polymers | Number of chains | 2 |
| Total formula weight | 105366.5 | |
| Non-Polymers* | Number of molecules | 13 |
| Total formula weight | 2854.1 | |
| All* | Total formula weight | 108220.7 |






