5J5Z

Crystal structure of the D444V disease-causing 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	Dihydrolipoyl dehydrogenase, mitochondrial	polymer	496	52621.2	2	UniProt (P09622) Pfam (PF07992) Pfam (PF02852) In PDB	Homo sapiens (Human)	Dihydrolipoamide dehydrogenase,Glycine cleavage system L protein
2	A, B	FLAVIN-ADENINE DINUCLEOTIDE	non-polymer		785.5	2	Chemie (FAD)
3	A, B	PHOSPHATE ION	non-polymer		95.0	2	Chemie (PO4)
4	A	GLYCEROL	non-polymer		92.1	3	Chemie (GOL)
5		water	water		18.0	495	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
Val 444	Asp 479	engineered mutation

Contents of the asymmetric unit

Polymers	Number of chains	2
	Total formula weight	105242.4
Non-Polymers*	Number of molecules	7
	Total formula weight	2037.3
All*	Total formula weight	107279.8

*Water molecules are not included.