6CER
Human pyruvate dehydrogenase complex E1 component V138M mutation
Functional Information from GO Data
| Chain | GOid | namespace | contents |
| A | 0004739 | molecular_function | pyruvate dehydrogenase (acetyl-transferring) activity |
| A | 0005515 | molecular_function | protein binding |
| A | 0005634 | cellular_component | nucleus |
| A | 0005739 | cellular_component | mitochondrion |
| A | 0005759 | cellular_component | mitochondrial matrix |
| A | 0006006 | biological_process | glucose metabolic process |
| A | 0006086 | biological_process | pyruvate decarboxylation to acetyl-CoA |
| A | 0006099 | biological_process | tricarboxylic acid cycle |
| A | 0016491 | molecular_function | oxidoreductase activity |
| A | 0016624 | molecular_function | oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor |
| A | 0032991 | cellular_component | protein-containing complex |
| A | 0043231 | cellular_component | intracellular membrane-bounded organelle |
| A | 0045254 | cellular_component | pyruvate dehydrogenase complex |
| A | 0046872 | molecular_function | metal ion binding |
| B | 0004739 | molecular_function | pyruvate dehydrogenase (acetyl-transferring) activity |
| B | 0005515 | molecular_function | protein binding |
| B | 0005634 | cellular_component | nucleus |
| B | 0005739 | cellular_component | mitochondrion |
| B | 0005759 | cellular_component | mitochondrial matrix |
| B | 0006006 | biological_process | glucose metabolic process |
| B | 0006086 | biological_process | pyruvate decarboxylation to acetyl-CoA |
| B | 0006099 | biological_process | tricarboxylic acid cycle |
| B | 0009060 | biological_process | aerobic respiration |
| B | 0016491 | molecular_function | oxidoreductase activity |
| B | 0044272 | biological_process | sulfur compound biosynthetic process |
| B | 0045254 | cellular_component | pyruvate dehydrogenase complex |
| B | 0046872 | molecular_function | metal ion binding |
| B | 0072522 | biological_process | purine-containing compound biosynthetic process |
| C | 0004739 | molecular_function | pyruvate dehydrogenase (acetyl-transferring) activity |
| C | 0005515 | molecular_function | protein binding |
| C | 0005634 | cellular_component | nucleus |
| C | 0005739 | cellular_component | mitochondrion |
| C | 0005759 | cellular_component | mitochondrial matrix |
| C | 0006006 | biological_process | glucose metabolic process |
| C | 0006086 | biological_process | pyruvate decarboxylation to acetyl-CoA |
| C | 0006099 | biological_process | tricarboxylic acid cycle |
| C | 0016491 | molecular_function | oxidoreductase activity |
| C | 0016624 | molecular_function | oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor |
| C | 0032991 | cellular_component | protein-containing complex |
| C | 0043231 | cellular_component | intracellular membrane-bounded organelle |
| C | 0045254 | cellular_component | pyruvate dehydrogenase complex |
| C | 0046872 | molecular_function | metal ion binding |
| D | 0004739 | molecular_function | pyruvate dehydrogenase (acetyl-transferring) activity |
| D | 0005515 | molecular_function | protein binding |
| D | 0005634 | cellular_component | nucleus |
| D | 0005739 | cellular_component | mitochondrion |
| D | 0005759 | cellular_component | mitochondrial matrix |
| D | 0006006 | biological_process | glucose metabolic process |
| D | 0006086 | biological_process | pyruvate decarboxylation to acetyl-CoA |
| D | 0006099 | biological_process | tricarboxylic acid cycle |
| D | 0009060 | biological_process | aerobic respiration |
| D | 0016491 | molecular_function | oxidoreductase activity |
| D | 0044272 | biological_process | sulfur compound biosynthetic process |
| D | 0045254 | cellular_component | pyruvate dehydrogenase complex |
| D | 0046872 | molecular_function | metal ion binding |
| D | 0072522 | biological_process | purine-containing compound biosynthetic process |
| E | 0004739 | molecular_function | pyruvate dehydrogenase (acetyl-transferring) activity |
| E | 0005515 | molecular_function | protein binding |
| E | 0005634 | cellular_component | nucleus |
| E | 0005739 | cellular_component | mitochondrion |
| E | 0005759 | cellular_component | mitochondrial matrix |
| E | 0006006 | biological_process | glucose metabolic process |
| E | 0006086 | biological_process | pyruvate decarboxylation to acetyl-CoA |
| E | 0006099 | biological_process | tricarboxylic acid cycle |
| E | 0016491 | molecular_function | oxidoreductase activity |
| E | 0016624 | molecular_function | oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor |
| E | 0032991 | cellular_component | protein-containing complex |
| E | 0043231 | cellular_component | intracellular membrane-bounded organelle |
| E | 0045254 | cellular_component | pyruvate dehydrogenase complex |
| E | 0046872 | molecular_function | metal ion binding |
| F | 0004739 | molecular_function | pyruvate dehydrogenase (acetyl-transferring) activity |
| F | 0005515 | molecular_function | protein binding |
| F | 0005634 | cellular_component | nucleus |
| F | 0005739 | cellular_component | mitochondrion |
| F | 0005759 | cellular_component | mitochondrial matrix |
| F | 0006006 | biological_process | glucose metabolic process |
| F | 0006086 | biological_process | pyruvate decarboxylation to acetyl-CoA |
| F | 0006099 | biological_process | tricarboxylic acid cycle |
| F | 0009060 | biological_process | aerobic respiration |
| F | 0016491 | molecular_function | oxidoreductase activity |
| F | 0044272 | biological_process | sulfur compound biosynthetic process |
| F | 0045254 | cellular_component | pyruvate dehydrogenase complex |
| F | 0046872 | molecular_function | metal ion binding |
| F | 0072522 | biological_process | purine-containing compound biosynthetic process |
| G | 0004739 | molecular_function | pyruvate dehydrogenase (acetyl-transferring) activity |
| G | 0005515 | molecular_function | protein binding |
| G | 0005634 | cellular_component | nucleus |
| G | 0005739 | cellular_component | mitochondrion |
| G | 0005759 | cellular_component | mitochondrial matrix |
| G | 0006006 | biological_process | glucose metabolic process |
| G | 0006086 | biological_process | pyruvate decarboxylation to acetyl-CoA |
| G | 0006099 | biological_process | tricarboxylic acid cycle |
| G | 0016491 | molecular_function | oxidoreductase activity |
| G | 0016624 | molecular_function | oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor |
| G | 0032991 | cellular_component | protein-containing complex |
| G | 0043231 | cellular_component | intracellular membrane-bounded organelle |
| G | 0045254 | cellular_component | pyruvate dehydrogenase complex |
| G | 0046872 | molecular_function | metal ion binding |
| H | 0004739 | molecular_function | pyruvate dehydrogenase (acetyl-transferring) activity |
| H | 0005515 | molecular_function | protein binding |
| H | 0005634 | cellular_component | nucleus |
| H | 0005739 | cellular_component | mitochondrion |
| H | 0005759 | cellular_component | mitochondrial matrix |
| H | 0006006 | biological_process | glucose metabolic process |
| H | 0006086 | biological_process | pyruvate decarboxylation to acetyl-CoA |
| H | 0006099 | biological_process | tricarboxylic acid cycle |
| H | 0009060 | biological_process | aerobic respiration |
| H | 0016491 | molecular_function | oxidoreductase activity |
| H | 0044272 | biological_process | sulfur compound biosynthetic process |
| H | 0045254 | cellular_component | pyruvate dehydrogenase complex |
| H | 0046872 | molecular_function | metal ion binding |
| H | 0072522 | biological_process | purine-containing compound biosynthetic process |
Functional Information from PDB Data
| site_id | AC1 |
| Number of Residues | 13 |
| Details | binding site for residue TPP A 401 |
| Chain | Residue |
| A | HIS63 |
| D | ILE57 |
| D | GLU59 |
| D | MET81 |
| D | PHE85 |
| A | TYR89 |
| A | ARG90 |
| A | GLY136 |
| A | MET138 |
| A | ARG259 |
| A | HOH503 |
| D | GLU28 |
| D | GLU29 |
| site_id | AC2 |
| Number of Residues | 11 |
| Details | binding site for residue TPP C 401 |
| Chain | Residue |
| B | GLU28 |
| B | ILE57 |
| B | GLU59 |
| B | MET81 |
| B | PHE85 |
| B | GLN88 |
| C | TYR89 |
| C | ARG90 |
| C | GLY136 |
| C | MET138 |
| C | HOH510 |
| site_id | AC3 |
| Number of Residues | 13 |
| Details | binding site for residue TPP E 401 |
| Chain | Residue |
| E | TYR89 |
| E | ARG90 |
| E | GLY136 |
| E | MET138 |
| E | ARG259 |
| E | MG402 |
| H | GLU28 |
| H | GLU29 |
| H | ILE57 |
| H | GLU59 |
| H | MET81 |
| H | PHE85 |
| H | GLN88 |
| site_id | AC4 |
| Number of Residues | 3 |
| Details | binding site for residue MG E 402 |
| Chain | Residue |
| E | ARG90 |
| E | ARG259 |
| E | TPP401 |
| site_id | AC5 |
| Number of Residues | 11 |
| Details | binding site for residue TPP G 401 |
| Chain | Residue |
| F | GLU28 |
| F | ILE57 |
| F | GLU59 |
| F | MET81 |
| F | PHE85 |
| G | HIS63 |
| G | TYR89 |
| G | ARG90 |
| G | GLY136 |
| G | MET138 |
| G | MG402 |
| site_id | AC6 |
| Number of Residues | 1 |
| Details | binding site for residue MG G 402 |
| Chain | Residue |
| G | TPP401 |
Functional Information from SwissProt/UniProt
| site_id | SWS_FT_FI1 |
| Number of Residues | 8 |
| Details | Binding site: {"evidences":[{"source":"PubMed","id":"29970614","evidenceCode":"ECO:0000305"},{"source":"PDB","id":"6CFO","evidenceCode":"ECO:0007744"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI2 |
| Number of Residues | 16 |
| Details | Binding 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"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI3 |
| Number of Residues | 4 |
| Details | Binding site: {"evidences":[{"source":"PubMed","id":"12651851","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"1NI4","evidenceCode":"ECO:0007744"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI4 |
| Number of Residues | 12 |
| Details | Binding 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"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI5 |
| Number of Residues | 11 |
| Details | Modified residue: {"description":"N6-succinyllysine; alternate","evidences":[{"source":"UniProtKB","id":"P35486","evidenceCode":"ECO:0000250"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI6 |
| Number of Residues | 8 |
| Details | Modified residue: {"description":"N6-succinyllysine","evidences":[{"source":"UniProtKB","id":"P35486","evidenceCode":"ECO:0000250"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI7 |
| Number of Residues | 4 |
| Details | Modified residue: {"description":"N6-acetyllysine","evidences":[{"source":"PubMed","id":"19608861","evidenceCode":"ECO:0007744"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI8 |
| Number of Residues | 4 |
| Details | Modified residue: {"description":"N6-acetyllysine","evidences":[{"source":"UniProtKB","id":"P35486","evidenceCode":"ECO:0000250"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI9 |
| Number of Residues | 4 |
| Details | Binding site: {"evidences":[{"source":"PubMed","id":"12651851","evidenceCode":"ECO:0000269"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI10 |
| Number of Residues | 4 |
| Details | Binding 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"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI11 |
| Number of Residues | 8 |
| Details | Binding 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"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI12 |
| Number of Residues | 4 |
| Details | Binding 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"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI13 |
| Number of Residues | 4 |
| Details | Binding 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"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI14 |
| Number of Residues | 4 |
| Details | Site: {"description":"Important for interaction with DLAT","evidences":[{"source":"PubMed","id":"20160912","evidenceCode":"ECO:0000269"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI15 |
| Number of Residues | 4 |
| Details | Modified residue: {"description":"Phosphotyrosine","evidences":[{"source":"UniProtKB","id":"Q9D051","evidenceCode":"ECO:0000250"}]} |
| Chain | Residue | Details |
| site_id | SWS_FT_FI16 |
| Number of Residues | 4 |
| Details | Modified residue: {"description":"N6-acetyllysine","evidences":[{"source":"UniProtKB","id":"Q9D051","evidenceCode":"ECO:0000250"}]} |
| Chain | Residue | Details |






