UniProt:Q9H4I9 SMDT1

chain
  • transit peptide:1-52
  • chain:53-107
checksum 4E00824D322AE99D
comment
  • 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, PubMed:30454562, PubMed:31080062, PubMed:32315830, PubMed:32494073, PubMed:32762847, PubMed:32790952, PubMed:33296646). Required to bridge the calcium-sensing proteins MICU1 with the calcium-conducting subunit MCU (PubMed:24231807, PubMed:30454562, PubMed:32494073, PubMed:32762847, PubMed:32790952). 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, PubMed:31080062, PubMed:32315830, PubMed:33296646).SUBUNIT Component of the uniplex complex, composed of MCU, EMRE/SMDT1, MICU1 and MICU2 (or MICU3) in a 4:4:1:1 stoichiometry (PubMed:24231807, PubMed:27099988, PubMed:31080062, PubMed:32315830, PubMed:32494073, PubMed:32762847, PubMed:32862359, PubMed:33296646). The number of EMRE/SMDT1 molecules is hovewer variable, ranging from 1 to 4 copies per uniplex complex, leading to uniplex complexes with distinct gatekeeping profiles (PubMed:32315830). Interacts (via its C-terminal poly-Asp tail) with MCUR1; the interaction is direct (PubMed:27184846). Unprocessed form interacts (via transit peptide) with MAIP1 (PubMed:27642048).INTERACTION 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).DOMAIN The GXXXX[G/A/S] motif at the C-terminal part of the transmembrane region mediates interaction with MCU and is required to activate the calcium-conducting pore in the uniporter complex.DOMAIN The poly-Asp region at the C-terminus mediates interaction with the polybasic region of MICU1.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 (PubMed:27642048, PubMed:28396416, PubMed:31097542).SIMILARITY Belongs to the SMDT1/EMRE family.CAUTION A publication reports an opposite topology (PubMed:26774479). However, 3 different articles, 2 in human and one in mouse, confirm the topology shown in this entry (PubMed:27099988, PubMed:27642048).
created [InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53
crossReference
databaseName UniProt
dbId 226174
description
  • recommendedName: fullName evidence="17"Essential MCU regulator, mitochondrial alternativeName: fullName evidence="23"Single-pass membrane protein with aspartate-rich tail 1, mitochondrial
displayName UniProt:Q9H4I9 SMDT1
geneName
  • SMDT1
  • C22orf32
  • EMRE
identifier Q9H4I9
isSequenceChanged false
keyword
  • 3D-structure
  • Calcium
  • Calcium transport
  • Ion transport
  • Membrane
  • Mitochondrion
  • Mitochondrion inner membrane
  • Proteomics identification
  • Reference proteome
  • Transit peptide
  • Transmembrane
  • Transmembrane helix
  • Transport
modified [InstanceEdit:9926675] Weiser, Joel, 2024-11-03
moleculeType Protein
name
  • SMDT1
otherIdentifier
  • 11729147_a_at
  • 11729148_a_at
  • 16930860
  • 225794_PM_s_at
  • 225794_s_at
  • 225795_PM_at
  • 225795_at
  • 227648_PM_at
  • 227648_at
  • 228117_PM_at
  • 228117_at
  • 3947311
  • 3947312
  • 3947313
  • 3947314
  • 3947316
  • 3947317
  • 3947318
  • 3947319
  • 3947320
  • 3947321
  • 3947322
  • 3947334
  • 3947335
  • 3947336
  • 3947337
  • 3947338
  • 3947341
  • 3947343
  • 42976_g_at
  • 42977_at
  • 57173_at
  • 77862_at
  • 8073578
  • 91689
  • A_14_P134946
  • A_21_P0014051
  • A_23_P211584
  • A_24_P271542
  • A_24_P753454
  • A_33_P3386508
  • A_33_P3440264
  • GE83641
  • GO:0005515
  • GO:0005654
  • GO:0005739
  • GO:0005743
  • GO:0005759
  • GO:0006811
  • GO:0006816
  • GO:0006851
  • GO:0016020
  • GO:0030674
  • GO:0036444
  • GO:0051560
  • GO:0055085
  • GO:0060090
  • GO:0071277
  • GO:0072732
  • GO:0099103
  • GO:1990246
  • HMNXSV003018518
  • HMNXSV003018846
  • HMNXSV003058214
  • Hs.218619.0.A1_3p_at
  • Hs.306083.0.S1_3p_a_at
  • ILMN_1706859
  • PH_hs_0015719
  • TC22001473.hg
  • p11215
  • p11216
  • p26194
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • EMRE_HUMAN
  • B2R5D1
  • Q8TAB9
sequenceLength 107
species [Species:48887] Homo sapiens
stId uniprot:Q9H4I9
url http://purl.uniprot.org/uniprot/Q9H4I9
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