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8GJU

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

Functional Information from GO Data
ChainGOidnamespacecontents
A0003924molecular_functionGTPase activity
A0005525molecular_functionGTP binding
B0003924molecular_functionGTPase activity
B0005525molecular_functionGTP binding
D0003924molecular_functionGTPase activity
D0005525molecular_functionGTP binding
F0003924molecular_functionGTPase activity
F0005525molecular_functionGTP binding
H0003824molecular_functioncatalytic activity
H0003924molecular_functionGTPase activity
H0004494molecular_functionmethylmalonyl-CoA mutase activity
H0005515molecular_functionprotein binding
H0005737cellular_componentcytoplasm
H0005739cellular_componentmitochondrion
H0005759cellular_componentmitochondrial matrix
H0006790biological_processsulfur compound metabolic process
H0016853molecular_functionisomerase activity
H0016866molecular_functionintramolecular transferase activity
H0019678biological_processpropionate metabolic process, methylmalonyl pathway
H0031419molecular_functioncobalamin binding
H0042802molecular_functionidentical protein binding
H0042803molecular_functionprotein homodimerization activity
H0043547biological_processpositive regulation of GTPase activity
H0044281biological_processsmall molecule metabolic process
H0046872molecular_functionmetal ion binding
H0050667biological_processhomocysteine metabolic process
H0072341molecular_functionmodified amino acid binding
H1901290biological_processsuccinyl-CoA biosynthetic process
J0003824molecular_functioncatalytic activity
J0003924molecular_functionGTPase activity
J0004494molecular_functionmethylmalonyl-CoA mutase activity
J0005515molecular_functionprotein binding
J0005737cellular_componentcytoplasm
J0005739cellular_componentmitochondrion
J0005759cellular_componentmitochondrial matrix
J0006790biological_processsulfur compound metabolic process
J0016853molecular_functionisomerase activity
J0016866molecular_functionintramolecular transferase activity
J0019678biological_processpropionate metabolic process, methylmalonyl pathway
J0031419molecular_functioncobalamin binding
J0042802molecular_functionidentical protein binding
J0042803molecular_functionprotein homodimerization activity
J0043547biological_processpositive regulation of GTPase activity
J0044281biological_processsmall molecule metabolic process
J0046872molecular_functionmetal ion binding
J0050667biological_processhomocysteine metabolic process
J0072341molecular_functionmodified amino acid binding
J1901290biological_processsuccinyl-CoA biosynthetic process
K0003824molecular_functioncatalytic activity
K0003924molecular_functionGTPase activity
K0004494molecular_functionmethylmalonyl-CoA mutase activity
K0005515molecular_functionprotein binding
K0005737cellular_componentcytoplasm
K0005739cellular_componentmitochondrion
K0005759cellular_componentmitochondrial matrix
K0006790biological_processsulfur compound metabolic process
K0016853molecular_functionisomerase activity
K0016866molecular_functionintramolecular transferase activity
K0019678biological_processpropionate metabolic process, methylmalonyl pathway
K0031419molecular_functioncobalamin binding
K0042802molecular_functionidentical protein binding
K0042803molecular_functionprotein homodimerization activity
K0043547biological_processpositive regulation of GTPase activity
K0044281biological_processsmall molecule metabolic process
K0046872molecular_functionmetal ion binding
K0050667biological_processhomocysteine metabolic process
K0072341molecular_functionmodified amino acid binding
K1901290biological_processsuccinyl-CoA biosynthetic process
L0003824molecular_functioncatalytic activity
L0003924molecular_functionGTPase activity
L0004494molecular_functionmethylmalonyl-CoA mutase activity
L0005515molecular_functionprotein binding
L0005737cellular_componentcytoplasm
L0005739cellular_componentmitochondrion
L0005759cellular_componentmitochondrial matrix
L0006790biological_processsulfur compound metabolic process
L0016853molecular_functionisomerase activity
L0016866molecular_functionintramolecular transferase activity
L0019678biological_processpropionate metabolic process, methylmalonyl pathway
L0031419molecular_functioncobalamin binding
L0042802molecular_functionidentical protein binding
L0042803molecular_functionprotein homodimerization activity
L0043547biological_processpositive regulation of GTPase activity
L0044281biological_processsmall molecule metabolic process
L0046872molecular_functionmetal ion binding
L0050667biological_processhomocysteine metabolic process
L0072341molecular_functionmodified amino acid binding
L1901290biological_processsuccinyl-CoA biosynthetic process
Functional Information from PROSITE/UniProt
site_idPS00544
Number of Residues26
DetailsMETMALONYL_COA_MUTASE Methylmalonyl-CoA mutase signature. RIARNTqiIIqEEsgipKvaDPwGGS
ChainResidueDetails
JARG403-SER428

Functional Information from SwissProt/UniProt
site_idSWS_FT_FI1
Number of Residues100
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"20876572","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails

site_idSWS_FT_FI2
Number of Residues4
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"20876572","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"28497574","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails

site_idSWS_FT_FI3
Number of Residues16
DetailsModified residue: {"description":"N6-acetyllysine","evidences":[{"source":"UniProtKB","id":"P16332","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails

site_idSWS_FT_FI4
Number of Residues6
DetailsModified residue: {"description":"N6-succinyllysine","evidences":[{"source":"UniProtKB","id":"P16332","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails

site_idSWS_FT_FI5
Number of Residues4
DetailsModified residue: {"description":"Phosphoserine","evidences":[{"source":"PubMed","id":"24275569","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

248636

PDB entries from 2026-02-04

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