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6CER

Human pyruvate dehydrogenase complex E1 component V138M mutation

Functional Information from GO Data
ChainGOidnamespacecontents
A0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
A0005515molecular_functionprotein binding
A0005634cellular_componentnucleus
A0005739cellular_componentmitochondrion
A0005759cellular_componentmitochondrial matrix
A0006006biological_processglucose metabolic process
A0006086biological_processpyruvate decarboxylation to acetyl-CoA
A0006099biological_processtricarboxylic acid cycle
A0016491molecular_functionoxidoreductase activity
A0016624molecular_functionoxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
A0032991cellular_componentprotein-containing complex
A0043231cellular_componentintracellular membrane-bounded organelle
A0045254cellular_componentpyruvate dehydrogenase complex
A0046872molecular_functionmetal ion binding
B0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
B0005515molecular_functionprotein binding
B0005634cellular_componentnucleus
B0005739cellular_componentmitochondrion
B0005759cellular_componentmitochondrial matrix
B0006006biological_processglucose metabolic process
B0006086biological_processpyruvate decarboxylation to acetyl-CoA
B0006099biological_processtricarboxylic acid cycle
B0009060biological_processaerobic respiration
B0016491molecular_functionoxidoreductase activity
B0044272biological_processsulfur compound biosynthetic process
B0045254cellular_componentpyruvate dehydrogenase complex
B0046872molecular_functionmetal ion binding
B0072522biological_processpurine-containing compound biosynthetic process
C0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
C0005515molecular_functionprotein binding
C0005634cellular_componentnucleus
C0005739cellular_componentmitochondrion
C0005759cellular_componentmitochondrial matrix
C0006006biological_processglucose metabolic process
C0006086biological_processpyruvate decarboxylation to acetyl-CoA
C0006099biological_processtricarboxylic acid cycle
C0016491molecular_functionoxidoreductase activity
C0016624molecular_functionoxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
C0032991cellular_componentprotein-containing complex
C0043231cellular_componentintracellular membrane-bounded organelle
C0045254cellular_componentpyruvate dehydrogenase complex
C0046872molecular_functionmetal ion binding
D0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
D0005515molecular_functionprotein binding
D0005634cellular_componentnucleus
D0005739cellular_componentmitochondrion
D0005759cellular_componentmitochondrial matrix
D0006006biological_processglucose metabolic process
D0006086biological_processpyruvate decarboxylation to acetyl-CoA
D0006099biological_processtricarboxylic acid cycle
D0009060biological_processaerobic respiration
D0016491molecular_functionoxidoreductase activity
D0044272biological_processsulfur compound biosynthetic process
D0045254cellular_componentpyruvate dehydrogenase complex
D0046872molecular_functionmetal ion binding
D0072522biological_processpurine-containing compound biosynthetic process
E0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
E0005515molecular_functionprotein binding
E0005634cellular_componentnucleus
E0005739cellular_componentmitochondrion
E0005759cellular_componentmitochondrial matrix
E0006006biological_processglucose metabolic process
E0006086biological_processpyruvate decarboxylation to acetyl-CoA
E0006099biological_processtricarboxylic acid cycle
E0016491molecular_functionoxidoreductase activity
E0016624molecular_functionoxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
E0032991cellular_componentprotein-containing complex
E0043231cellular_componentintracellular membrane-bounded organelle
E0045254cellular_componentpyruvate dehydrogenase complex
E0046872molecular_functionmetal ion binding
F0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
F0005515molecular_functionprotein binding
F0005634cellular_componentnucleus
F0005739cellular_componentmitochondrion
F0005759cellular_componentmitochondrial matrix
F0006006biological_processglucose metabolic process
F0006086biological_processpyruvate decarboxylation to acetyl-CoA
F0006099biological_processtricarboxylic acid cycle
F0009060biological_processaerobic respiration
F0016491molecular_functionoxidoreductase activity
F0044272biological_processsulfur compound biosynthetic process
F0045254cellular_componentpyruvate dehydrogenase complex
F0046872molecular_functionmetal ion binding
F0072522biological_processpurine-containing compound biosynthetic process
G0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
G0005515molecular_functionprotein binding
G0005634cellular_componentnucleus
G0005739cellular_componentmitochondrion
G0005759cellular_componentmitochondrial matrix
G0006006biological_processglucose metabolic process
G0006086biological_processpyruvate decarboxylation to acetyl-CoA
G0006099biological_processtricarboxylic acid cycle
G0016491molecular_functionoxidoreductase activity
G0016624molecular_functionoxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
G0032991cellular_componentprotein-containing complex
G0043231cellular_componentintracellular membrane-bounded organelle
G0045254cellular_componentpyruvate dehydrogenase complex
G0046872molecular_functionmetal ion binding
H0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
H0005515molecular_functionprotein binding
H0005634cellular_componentnucleus
H0005739cellular_componentmitochondrion
H0005759cellular_componentmitochondrial matrix
H0006006biological_processglucose metabolic process
H0006086biological_processpyruvate decarboxylation to acetyl-CoA
H0006099biological_processtricarboxylic acid cycle
H0009060biological_processaerobic respiration
H0016491molecular_functionoxidoreductase activity
H0044272biological_processsulfur compound biosynthetic process
H0045254cellular_componentpyruvate dehydrogenase complex
H0046872molecular_functionmetal ion binding
H0072522biological_processpurine-containing compound biosynthetic process
Functional Information from PDB Data
site_idAC1
Number of Residues13
Detailsbinding site for residue TPP A 401
ChainResidue
AHIS63
DILE57
DGLU59
DMET81
DPHE85
ATYR89
AARG90
AGLY136
AMET138
AARG259
AHOH503
DGLU28
DGLU29

site_idAC2
Number of Residues11
Detailsbinding site for residue TPP C 401
ChainResidue
BGLU28
BILE57
BGLU59
BMET81
BPHE85
BGLN88
CTYR89
CARG90
CGLY136
CMET138
CHOH510

site_idAC3
Number of Residues13
Detailsbinding site for residue TPP E 401
ChainResidue
ETYR89
EARG90
EGLY136
EMET138
EARG259
EMG402
HGLU28
HGLU29
HILE57
HGLU59
HMET81
HPHE85
HGLN88

site_idAC4
Number of Residues3
Detailsbinding site for residue MG E 402
ChainResidue
EARG90
EARG259
ETPP401

site_idAC5
Number of Residues11
Detailsbinding site for residue TPP G 401
ChainResidue
FGLU28
FILE57
FGLU59
FMET81
FPHE85
GHIS63
GTYR89
GARG90
GGLY136
GMET138
GMG402

site_idAC6
Number of Residues1
Detailsbinding site for residue MG G 402
ChainResidue
GTPP401

Functional Information from SwissProt/UniProt
site_idSWS_FT_FI1
Number of Residues8
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"29970614","evidenceCode":"ECO:0000305"},{"source":"PDB","id":"6CFO","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

site_idSWS_FT_FI2
Number of Residues16
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"12651851","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"17474719","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"19081061","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"29970614","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"1NI4","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"2OZL","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXE","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXF","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXH","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"6CER","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

site_idSWS_FT_FI3
Number of Residues4
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"12651851","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"1NI4","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

site_idSWS_FT_FI4
Number of Residues12
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"12651851","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"17474719","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"19081061","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"1NI4","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"2OZL","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXE","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXF","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXH","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

site_idSWS_FT_FI5
Number of Residues11
DetailsModified residue: {"description":"N6-succinyllysine; alternate","evidences":[{"source":"UniProtKB","id":"P35486","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails

site_idSWS_FT_FI6
Number of Residues8
DetailsModified residue: {"description":"N6-succinyllysine","evidences":[{"source":"UniProtKB","id":"P35486","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails

site_idSWS_FT_FI7
Number of Residues4
DetailsModified residue: {"description":"N6-acetyllysine","evidences":[{"source":"PubMed","id":"19608861","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

site_idSWS_FT_FI8
Number of Residues4
DetailsModified residue: {"description":"N6-acetyllysine","evidences":[{"source":"UniProtKB","id":"P35486","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails

site_idSWS_FT_FI9
Number of Residues4
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"12651851","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails

site_idSWS_FT_FI10
Number of Residues4
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"12651851","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"17474719","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"1NI4","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"2OZL","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

site_idSWS_FT_FI11
Number of Residues8
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"12651851","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"17474719","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"19081061","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"1NI4","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"2OZL","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXE","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXF","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXG","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXH","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXI","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

site_idSWS_FT_FI12
Number of Residues4
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"19081061","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"3EXE","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXF","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXG","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXH","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXI","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

site_idSWS_FT_FI13
Number of Residues4
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"12651851","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"17474719","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"19081061","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"1NI4","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"2OZL","evidenceCode":"ECO:0007744"},{"source":"PDB","id":"3EXE","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

site_idSWS_FT_FI14
Number of Residues4
DetailsSite: {"description":"Important for interaction with DLAT","evidences":[{"source":"PubMed","id":"20160912","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails

site_idSWS_FT_FI15
Number of Residues4
DetailsModified residue: {"description":"Phosphotyrosine","evidences":[{"source":"UniProtKB","id":"Q9D051","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails

site_idSWS_FT_FI16
Number of Residues4
DetailsModified residue: {"description":"N6-acetyllysine","evidences":[{"source":"UniProtKB","id":"Q9D051","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails

246704

PDB entries from 2025-12-24

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