Detail Information for IndEnz0002019286
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