| IED ID | IndEnz0002019286 |
| Enzyme Type ID | protease019286 |
| Protein Name |
Essential MCU regulator, mitochondrial Single-pass membrane protein with aspartate-rich tail 1, mitochondrial |
| Gene Name | SMDT1 C22orf32 EMRE |
| 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 | MASGAARWLVLAPVRSGALRSGPSLRKDGDVSAAWSGSGRSLVPSRSVIVTRSGAILPKPVKMSFGLLRVFSIVIPFLYVGTLISKNFAALLEEHDIFVPEDDDDDD |
| Enzyme Length | 107 |
| Uniprot Accession Number | Q9H4I9 |
| Absorption | |
| Active Site | |
| Activity Regulation | |
| Binding Site | |
| Calcium Binding | |
| catalytic Activity | |
| DNA Binding | |
| EC Number | |
| Enzyme Function | FUNCTION: Essential regulatory subunit of the mitochondrial calcium uniporter complex (uniplex), a complex that mediates calcium uptake into mitochondria (PubMed:24231807, PubMed:26774479, PubMed:27099988). Required to bridge the calcium-sensing proteins MICU1 and MICU2 with the calcium-conducting subunit MCU (PubMed:24231807). Plays a central role in regulating the uniplex complex response to intracellular calcium signaling (PubMed:27099988). Acts by mediating activation of MCU and retention of MICU1 to the MCU pore, in order to ensure tight regulation of the uniplex complex and appropriate responses to intracellular calcium signaling (PubMed:27099988). {ECO:0000269|PubMed:24231807, ECO:0000269|PubMed:26774479, ECO:0000269|PubMed:27099988}. |
| Temperature Dependency | |
| PH Dependency | |
| Pathway | |
| nucleotide Binding | |
| Features | Beta strand (1); Chain (1); Helix (3); Motif (1); Mutagenesis (3); Natural variant (1); Region (1); Topological domain (2); Transit peptide (1); Transmembrane (1); Turn (1) |
| Keywords | 3D-structure;Calcium;Calcium transport;Ion transport;Membrane;Mitochondrion;Mitochondrion inner membrane;Reference proteome;Transit peptide;Transmembrane;Transmembrane helix;Transport |
| Interact With | Q8NE86 |
| Induction | |
| Subcellular Location | SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000269|PubMed:24231807, ECO:0000269|PubMed:26774479, ECO:0000269|PubMed:27099988, ECO:0000269|PubMed:27642048}; Single-pass membrane protein {ECO:0000269|PubMed:24231807, ECO:0000269|PubMed:27099988}. Note=MAIP1 is required to assist sorting of EMRE/SMDT1 into mitochondrion by protecting EMRE/SMDT1 against protein degradation by YME1L1, thereby ensuring SMDT1/EMRE maturation by the mitochondrial processing peptidase (PMPCA and PMPCB) (PubMed:27642048). {ECO:0000269|PubMed:27642048}. |
| Modified Residue | |
| Post Translational Modification | PTM: Undergoes proteolytic degradation in neurons: degraded by AFG3L2 before SMDT1/EMRE assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU (PubMed:27642048). {ECO:0000269|PubMed:27642048}. |
| Signal Peptide | |
| Structure 3D | Electron microscopy (8) |
| Cross Reference PDB | 6K7X; 6K7Y; 6O58; 6O5B; 6WDN; 6WDO; 6XJV; 6XJX; |
| Mapped Pubmed ID | 20693986; 20877624; 21685886; 21685888; 23409044; 23755363; 23900286; 24162235; 24503055; 24560927; 25565209; 25753332; 26489515; 26976564; 27737933; 30782485; 31080062; 32494073; 32762847; 32862359; 33296646; 7399049; |
| Motif | MOTIF 81..85; /note=GXXXX[G/A/S]; /evidence=ECO:0000305|PubMed:27099988 |
| Gene Encoded By | |
| Mass | 11,441 |
| Kinetics | |
| Metal Binding | |
| Rhea ID | |
| Cross Reference Brenda |