Essential MCU regulator, mitochondrial {ECO:0000303|PubMed:24231807}

UniProt data

UniProt name: EMRE_HUMAN (Q9H4I9)

Gene name: SMDT1 {ECO:0000312|HGNC:HGNC:25055}

Organism: Homo sapiens (Human).

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}.

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=C2orf47/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}.

Transmembrane domains:

  1. 65-84, 20 AA, Helical. {ECO:0000255}.

Sequence: 107 AA

10 20 30 40 50 60 MASGAARWLV LAPVRSGALR SGPSLRKDGD VSAAWSGSGR SLVPSRSVIV TRSGAILPKP 70 80 90 100 VKMSFGLLRV FSIVIPFLYV GTLISKNFAA LLEEHDIFVP EDDDDDD

CATM prediction

The sequence of EMRE_HUMAN returned 1 potential models

The positions at the dimer's interface are highlighted in yellow. The position at the crossing point is highlighted in orange.

# Sequence and interface d θ Z' ω' Score
          70        80      
   +    |    +    |    +  		 (Å) (°) (Å) (°)  
Model 1 KMSFGLLRVFSIVIAFLYVGTLISKN 7.2 -51.1° 1.2 44.9 -19.1
Prolines* KMSFGLLRVFSIVIPFLYVGTLISKN          

* Note: this sequence contains Pro residues. CATM substituted them with Ala during the modeling. The positions are marked.

Click on the model number to visualize its details below.

MODEL 1 DETAILS

Precited structure:


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Download model 1 structure: PDB, PyMOL session

List of hydrogen bonds:

Donor Acceptor Distance
B, 81, GLY, HA1 A, 78, LEU, O 3.32Å
B, 82, THR, HA A, 81, GLY, O 3.44Å
A, 81, GLY, HA1 B, 78, LEU, O 3.32Å
A, 82, THR, HA B, 81, GLY, O 3.44Å