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6CER

Human pyruvate dehydrogenase complex E1 component V138M mutation

Functional Information from GO Data
ChainGOidnamespacecontents
A0004738molecular_functionpyruvate dehydrogenase activity
A0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
A0005515molecular_functionprotein binding
A0005634cellular_componentnucleus
A0005730cellular_componentnucleolus
A0005739cellular_componentmitochondrion
A0005759cellular_componentmitochondrial matrix
A0006006biological_processglucose metabolic process
A0006086biological_processacetyl-CoA biosynthetic process from pyruvate
A0006090biological_processpyruvate metabolic process
A0006099biological_processtricarboxylic acid cycle
A0016491molecular_functionoxidoreductase activity
A0016624molecular_functionoxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
A0034604molecular_functionpyruvate dehydrogenase (NAD+) activity
A0043231cellular_componentintracellular membrane-bounded organelle
A0045254cellular_componentpyruvate dehydrogenase complex
A1902494cellular_componentcatalytic complex
B0003824molecular_functioncatalytic activity
B0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
B0005515molecular_functionprotein binding
B0005634cellular_componentnucleus
B0005654cellular_componentnucleoplasm
B0005739cellular_componentmitochondrion
B0005759cellular_componentmitochondrial matrix
B0006006biological_processglucose metabolic process
B0006086biological_processacetyl-CoA biosynthetic process from pyruvate
B0006099biological_processtricarboxylic acid cycle
B0016491molecular_functionoxidoreductase activity
B0034604molecular_functionpyruvate dehydrogenase (NAD+) activity
B0045254cellular_componentpyruvate dehydrogenase complex
C0004738molecular_functionpyruvate dehydrogenase activity
C0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
C0005515molecular_functionprotein binding
C0005634cellular_componentnucleus
C0005730cellular_componentnucleolus
C0005739cellular_componentmitochondrion
C0005759cellular_componentmitochondrial matrix
C0006006biological_processglucose metabolic process
C0006086biological_processacetyl-CoA biosynthetic process from pyruvate
C0006090biological_processpyruvate metabolic process
C0006099biological_processtricarboxylic acid cycle
C0016491molecular_functionoxidoreductase activity
C0016624molecular_functionoxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
C0034604molecular_functionpyruvate dehydrogenase (NAD+) activity
C0043231cellular_componentintracellular membrane-bounded organelle
C0045254cellular_componentpyruvate dehydrogenase complex
C1902494cellular_componentcatalytic complex
D0003824molecular_functioncatalytic activity
D0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
D0005515molecular_functionprotein binding
D0005634cellular_componentnucleus
D0005654cellular_componentnucleoplasm
D0005739cellular_componentmitochondrion
D0005759cellular_componentmitochondrial matrix
D0006006biological_processglucose metabolic process
D0006086biological_processacetyl-CoA biosynthetic process from pyruvate
D0006099biological_processtricarboxylic acid cycle
D0016491molecular_functionoxidoreductase activity
D0034604molecular_functionpyruvate dehydrogenase (NAD+) activity
D0045254cellular_componentpyruvate dehydrogenase complex
E0004738molecular_functionpyruvate dehydrogenase activity
E0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
E0005515molecular_functionprotein binding
E0005634cellular_componentnucleus
E0005730cellular_componentnucleolus
E0005739cellular_componentmitochondrion
E0005759cellular_componentmitochondrial matrix
E0006006biological_processglucose metabolic process
E0006086biological_processacetyl-CoA biosynthetic process from pyruvate
E0006090biological_processpyruvate metabolic process
E0006099biological_processtricarboxylic acid cycle
E0016491molecular_functionoxidoreductase activity
E0016624molecular_functionoxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
E0034604molecular_functionpyruvate dehydrogenase (NAD+) activity
E0043231cellular_componentintracellular membrane-bounded organelle
E0045254cellular_componentpyruvate dehydrogenase complex
E1902494cellular_componentcatalytic complex
F0003824molecular_functioncatalytic activity
F0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
F0005515molecular_functionprotein binding
F0005634cellular_componentnucleus
F0005654cellular_componentnucleoplasm
F0005739cellular_componentmitochondrion
F0005759cellular_componentmitochondrial matrix
F0006006biological_processglucose metabolic process
F0006086biological_processacetyl-CoA biosynthetic process from pyruvate
F0006099biological_processtricarboxylic acid cycle
F0016491molecular_functionoxidoreductase activity
F0034604molecular_functionpyruvate dehydrogenase (NAD+) activity
F0045254cellular_componentpyruvate dehydrogenase complex
G0004738molecular_functionpyruvate dehydrogenase activity
G0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
G0005515molecular_functionprotein binding
G0005634cellular_componentnucleus
G0005730cellular_componentnucleolus
G0005739cellular_componentmitochondrion
G0005759cellular_componentmitochondrial matrix
G0006006biological_processglucose metabolic process
G0006086biological_processacetyl-CoA biosynthetic process from pyruvate
G0006090biological_processpyruvate metabolic process
G0006099biological_processtricarboxylic acid cycle
G0016491molecular_functionoxidoreductase activity
G0016624molecular_functionoxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
G0034604molecular_functionpyruvate dehydrogenase (NAD+) activity
G0043231cellular_componentintracellular membrane-bounded organelle
G0045254cellular_componentpyruvate dehydrogenase complex
G1902494cellular_componentcatalytic complex
H0003824molecular_functioncatalytic activity
H0004739molecular_functionpyruvate dehydrogenase (acetyl-transferring) activity
H0005515molecular_functionprotein binding
H0005634cellular_componentnucleus
H0005654cellular_componentnucleoplasm
H0005739cellular_componentmitochondrion
H0005759cellular_componentmitochondrial matrix
H0006006biological_processglucose metabolic process
H0006086biological_processacetyl-CoA biosynthetic process from pyruvate
H0006099biological_processtricarboxylic acid cycle
H0016491molecular_functionoxidoreductase activity
H0034604molecular_functionpyruvate dehydrogenase (NAD+) activity
H0045254cellular_componentpyruvate dehydrogenase complex
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 Residues4
DetailsBINDING: BINDING => ECO:0000269|PubMed:12651851
ChainResidueDetails
BGLU59
GLYS34
GLYS215
GLYS284
DGLU59
FGLU59
HGLU59
CLYS215
CLYS284
ELYS34
ELYS215
ELYS284

site_idSWS_FT_FI2
Number of Residues4
DetailsSITE: Important for interaction with DLAT => ECO:0000269|PubMed:20160912
ChainResidueDetails
BASP289
DASP289
FASP289
HASP289

site_idSWS_FT_FI3
Number of Residues4
DetailsMOD_RES: Phosphotyrosine => ECO:0000250|UniProtKB:Q9D051
ChainResidueDetails
BTYR37
DTYR37
FTYR37
HTYR37
ELYS248
ELYS356
GLYS248
GLYS356

site_idSWS_FT_FI4
Number of Residues4
DetailsMOD_RES: N6-acetyllysine => ECO:0000250|UniProtKB:Q9D051
ChainResidueDetails
BLYS324
DLYS324
FLYS324
HLYS324

site_idSWS_FT_FI5
Number of Residues4
DetailsMOD_RES: Phosphoserine => ECO:0000250|UniProtKB:P35486
ChainResidueDetails
ASER266
CSER266
ESER266
GSER266

site_idSWS_FT_FI6
Number of Residues4
DetailsMOD_RES: Phosphoserine; by PDK1, PDK2, PDK3 and PDK4 => ECO:0000269|PubMed:11486000, ECO:0000269|PubMed:19081061
ChainResidueDetails
ASER271
CSER271
ESER271
GSER271

site_idSWS_FT_FI7
Number of Residues4
DetailsMOD_RES: Phosphotyrosine => ECO:0000250|UniProtKB:P35486
ChainResidueDetails
ATYR272
CTYR272
ETYR272
GTYR272

site_idSWS_FT_FI8
Number of Residues4
DetailsMOD_RES: N6-acetyllysine => ECO:0007744|PubMed:19608861
ChainResidueDetails
ALYS292
CLYS292
ELYS292
GLYS292

site_idSWS_FT_FI9
Number of Residues4
DetailsMOD_RES: N6-acetyllysine => ECO:0000250|UniProtKB:P35486
ChainResidueDetails
ALYS307
CLYS307
ELYS307
GLYS307

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PDB entries from 2024-07-24

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