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	A, B, F, G (D, F, A, B)	Methylmalonic aciduria type A protein, mitochondrial	polymer	349	38718.9	4	UniProt (Q8IVH4) Pfam (PF03308)	Homo sapiens (human)
2	C, D, E, H (J, K, L, H)	Methylmalonyl-CoA mutase, mitochondrial	polymer	748	83010.0	4	UniProt (A0A2S1PH20) Pfam (PF01642) Pfam (PF02310) UniProt (by SIFTS) (P22033)	Homo sapiens (human)	Methylmalonyl-CoA isomerase
3	I, K, P, R (D, F, A, B)	GUANOSINE-5'-DIPHOSPHATE	non-polymer		443.2	4	Chemie (GDP) PubChem (136073927) PubChem (135398619) PubChem (135402030) PubChem (135431654) PubChem (135566337) PubChem (135760556) PubChem (136026707) PubChem (136053418) PubChem (136663964) PubChem (136664748) PubChem (136759848) PubChem (136759849) PubChem (136759850) PubChem (136836636) PubChem (136847074) PubChem (172898537)
4	J, L, Q, S (D, F, A, B)	MAGNESIUM ION	non-polymer		24.3	4	Chemie (MG) PubChem (888)
5	M, N, O, T (J, K, L, H)	COENZYME A	non-polymer		767.5	4	Chemie (COA)
6	U, V, W, X, Y (D, F, A, B, H)	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.