| IED ID | IndEnz0002009455 |
| Enzyme Type ID | protease009455 |
| Protein Name |
Transcription factor A, mitochondrial mtTFA Mitochondrial transcription factor 1 MtTF1 Transcription factor 6 TCF-6 Transcription factor 6-like 2 |
| Gene Name | TFAM TCF6 TCF6L2 |
| Organism | Homo sapiens (Human) |
| Taxonomic Lineage | cellular organisms Eukaryota Opisthokonta Metazoa Eumetazoa Bilateria Deuterostomia Chordata Craniata Vertebrata Gnathostomata (jawed vertebrates) Teleostomi Euteleostomi Sarcopterygii Dipnotetrapodomorpha Tetrapoda Amniota Mammalia Theria Eutheria Boreoeutheria Euarchontoglires Primates Haplorrhini Simiiformes Catarrhini Hominoidea (apes) Hominidae (great apes) Homininae Homo Homo sapiens (Human) |
| Enzyme Sequence | MAFLRSMWGVLSALGRSGAELCTGCGSRLRSPFSFVYLPRWFSSVLASCPKKPVSSYLRFSKEQLPIFKAQNPDAKTTELIRRIAQRWRELPDSKKKIYQDAYRAEWQVYKEEISRFKEQLTPSQIMSLEKEIMDKHLKRKAMTKKKELTLLGKPKRPRSAYNVYVAERFQEAKGDSPQEKLKTVKENWKNLSDSEKELYIQHAKEDETRYHNEMKSWEEQMIEVGRKDLLRRTIKKQRKYGAEEC |
| Enzyme Length | 246 |
| Uniprot Accession Number | Q00059 |
| Absorption | |
| Active Site | |
| Activity Regulation | |
| Binding Site | |
| Calcium Binding | |
| catalytic Activity | |
| DNA Binding | DNA_BIND 50..118; /note=HMG box 1; /evidence=ECO:0000255|PROSITE-ProRule:PRU00267; DNA_BIND 155..219; /note=HMG box 2; /evidence=ECO:0000255|PROSITE-ProRule:PRU00267 |
| EC Number | |
| Enzyme Function | FUNCTION: Binds to the mitochondrial light strand promoter and functions in mitochondrial transcription regulation (PubMed:29445193, PubMed:32183942). Component of the mitochondrial transcription initiation complex, composed at least of TFB2M, TFAM and POLRMT that is required for basal transcription of mitochondrial DNA (PubMed:29149603). In this complex, TFAM recruits POLRMT to a specific promoter whereas TFB2M induces structural changes in POLRMT to enable promoter opening and trapping of the DNA non-template strand (PubMed:20410300). Required for accurate and efficient promoter recognition by the mitochondrial RNA polymerase (PubMed:22037172). Promotes transcription initiation from the HSP1 and the light strand promoter by binding immediately upstream of transcriptional start sites (PubMed:22037172). Is able to unwind DNA (PubMed:22037172). Bends the mitochondrial light strand promoter DNA into a U-turn shape via its HMG boxes (PubMed:1737790). Required for maintenance of normal levels of mitochondrial DNA (PubMed:22841477, PubMed:19304746). May play a role in organizing and compacting mitochondrial DNA (PubMed:22037171). {ECO:0000269|PubMed:1737790, ECO:0000269|PubMed:19304746, ECO:0000269|PubMed:20410300, ECO:0000269|PubMed:22037171, ECO:0000269|PubMed:22037172, ECO:0000269|PubMed:22841477, ECO:0000269|PubMed:29149603, ECO:0000269|PubMed:29445193, ECO:0000269|PubMed:32183942}. |
| Temperature Dependency | |
| PH Dependency | |
| Pathway | |
| nucleotide Binding | |
| Features | Alternative sequence (1); Beta strand (2); Chain (1); DNA binding (2); Helix (9); Modified residue (7); Mutagenesis (2); Natural variant (2); Sequence conflict (1); Site (2); Transit peptide (1) |
| Keywords | 3D-structure;Activator;Alternative splicing;DNA-binding;Direct protein sequencing;Disease variant;Mitochondrion;Mitochondrion nucleoid;Phosphoprotein;Primary mitochondrial disease;Reference proteome;Repeat;Transcription;Transcription regulation;Transit peptide |
| Interact With | Q6RW13; Q6RW13-2; Q15041; Q4VAQ0; O14880; P02808; Q9NZ01; Q8WVM0; O60635 |
| Induction | |
| Subcellular Location | SUBCELLULAR LOCATION: Mitochondrion {ECO:0000269|PubMed:1737790, ECO:0000269|PubMed:18063578, ECO:0000269|PubMed:22841477, ECO:0000269|PubMed:29445193}. Mitochondrion matrix, mitochondrion nucleoid {ECO:0000269|PubMed:18063578}. |
| Modified Residue | MOD_RES 55; /note="Phosphoserine; by PKA"; /evidence="ECO:0000269|PubMed:23201127"; MOD_RES 56; /note="Phosphoserine; by PKA"; /evidence="ECO:0000269|PubMed:23201127"; MOD_RES 61; /note="Phosphoserine; by PKA"; /evidence="ECO:0000269|PubMed:23201127"; MOD_RES 122; /note="Phosphothreonine"; /evidence="ECO:0007744|PubMed:23186163"; MOD_RES 160; /note="Phosphoserine; by PKA"; /evidence="ECO:0000269|PubMed:23201127"; MOD_RES 193; /note="Phosphoserine"; /evidence="ECO:0007744|PubMed:18669648, ECO:0007744|PubMed:23186163"; MOD_RES 195; /note="Phosphoserine"; /evidence="ECO:0007744|PubMed:23186163" |
| Post Translational Modification | PTM: Phosphorylation by PKA within the HMG box 1 impairs DNA binding and promotes degradation by the AAA+ Lon protease. {ECO:0000269|PubMed:23201127}. |
| Signal Peptide | |
| Structure 3D | X-ray crystallography (9) |
| Cross Reference PDB | 3FGH; 3TMM; 3TQ6; 4NNU; 4NOD; 6ERP; 6ERQ; 6HB4; 6HC3; |
| Mapped Pubmed ID | 10412986; 11340167; 11522837; 12068295; 12930954; 15464268; 15526033; 16385451; 16911507; 17353931; 17537576; 18660489; 18847512; 19738201; 20186120; 21150319; 22623428; 22904065; 23902751; 24058615; 24435062; 25416956; 25609649; 25665578; 26058080; 28242328; 31114891; 32371956; 3594571; 8108407; 8333869; |
| Motif | |
| Gene Encoded By | |
| Mass | 29,097 |
| Kinetics | |
| Metal Binding | |
| Rhea ID | |
| Cross Reference Brenda |