Smdt1
entrez
69029
canonical
uniprot
Q9DB10
canonical
ensembl
ENSMUSG00000022452
resolver
ensp
ENSMUSP00000023086
resolver
enst
ENSMUST00000023086
resolver
genesymbol
Smdt1
resolver
genesymbol_syn
Emre
resolver
uniprot
Q58EA5
resolver
uniprot_entry
EMRE_MOUSE
resolver
uniprot_sec
Q58EA5
resolver
amino_acid_sequence
MASTAARRLAWVAVRPGALWSGPRGRRGGDVYTVPGSSGLSQVPSRSVIVTRSGAILPKPVKMSFGLLRVFSIVIPFLYVGTLISKNFAALLEEHDIFVPEDDDDDD
uniprot
proteins
function
FUNCTION: Essential regulatory subunit of the mitochondrial calcium uniporter complex (uniplex), a complex that mediates calcium uptake into mitochondria (PubMed:27001609). Required to bridge the calcium-sensing proteins MICU1 with the calcium-conducting subunit MCU (By similarity). 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 (By similarity). {ECO:0000250|UniProtKB:Q9H4I9, ECO:0000269|PubMed:27001609}.
uniprot
proteins
mass_dalton
11542
uniprot
proteins
mutagenesis
MUTAGEN 60; /note="P->A: Abolishes calcium uptake into mitochondria. Abolishes interaction with MCU."; /evidence="ECO:0000269|PubMed:27001609"; MUTAGEN 85..90; /note="Missing: Abolishes calcium uptake into mitochondria. Abolishes interaction with MCU."; /evidence="ECO:0000269|PubMed:27001609
uniprot
proteins
post_translational_modification
PTM: Undergoes proteolytic degradation in neurons: degraded by AFG3L2 and SPG7 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. {ECO:0000250|UniProtKB:Q9H4I9}.
uniprot
proteins
protein_family
SMDT1/EMRE family
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
24231807
uniprot
proteins
pubmed
27001609
uniprot
proteins
sequence_length
107
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000269|PubMed:27001609}; Single-pass membrane protein {ECO:0000250|UniProtKB:Q9H4I9}. 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). {ECO:0000250|UniProtKB:Q9H4I9}.
uniprot
proteins
transmembrane_region
TRANSMEM 66..85; /note="Helical"; /evidence="ECO:0000250|UniProtKB:Q9H4I9
uniprot
proteins