8GJU

Crystal structure of human methylmalonyl-CoA mutase (MMUT) in complex with methylmalonic acidemia type A protein (MMAA), coenzyme A, and GDP

Entity

Entity ID	Chain ID	Description	Type	Chain length	Formula weight	Number of molecules	DB Name (Accession)	Biological source	Descriptive keywords
1	D, F, A, B	Methylmalonic aciduria type A protein, mitochondrial	polymer	349	38718.9	4	UniProt (Q8IVH4) Pfam (PF03308) In PDB	Homo sapiens (human)
2	J, K, L, H	Methylmalonyl-CoA mutase, mitochondrial	polymer	748	83010.0	4	UniProt (A0A2S1PH20) Pfam (PF01642) Pfam (PF02310) UniProt (by SIFTS) (P22033) In PDB	Homo sapiens (human)	Methylmalonyl-CoA isomerase
3	D, F, A, B	GUANOSINE-5'-DIPHOSPHATE	non-polymer		443.2	4	Chemie (GDP)
4	D, F, A, B	MAGNESIUM ION	non-polymer		24.3	4	Chemie (MG)
5	J, K, L, H	COENZYME A	non-polymer		767.5	4	Chemie (COA)
6		water	water		18.0	13	Chemie (HOH)

Sequence modifications

D, F, A, B: 72 - 418 (UniProt: Q8IVH4)

PDB	External Database	Details
Ser 70	-	expression tag
Met 71	-	expression tag

J, K, L, H: 12 - 750 (UniProt: A0A2S1PH20)

PDB	External Database	Details
Met 11	-	initiating methionine
Thr 499	Ala 499	conflict
Ala 751	-	expression tag
Glu 752	-	expression tag
Asn 753	-	expression tag
Leu 754	-	expression tag
Tyr 755	-	expression tag
Phe 756	-	expression tag
Gln 757	-	expression tag
Ser 758	-	expression tag

Contents of the asymmetric unit

Polymers	Number of chains	8
	Total formula weight	486915.3
Non-Polymers*	Number of molecules	12
	Total formula weight	4940.2
All*	Total formula weight	491855.5

*Water molecules are not included.