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Details on Person UniProt:Q9H4I9 SMDT1
| Class:Id | ReferenceGeneProduct:226174 |
|---|---|
| _chainChangeLog | transit peptide:1-47 added on Fri February 6 2015;chain:48-107 added on Fri February 6 2015;transit peptide:1-47 removed on Fri November 4 2016;chain:48-107 removed on Fri November 4 2016;transit peptide:1-52 added on Fri November 4 2016;chain:53-107 added on Fri November 4 2016 |
| _displayName | UniProt:Q9H4I9 SMDT1 |
| _timestamp | 2024-11-03 19:51:04 |
| 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 |
| description | recommendedName: fullName evidence="17"Essential MCU regulator, mitochondrial alternativeName: fullName evidence="23"Single-pass membrane protein with aspartate-rich tail 1, mitochondrial |
| 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:354386] Schmidt, EE, 2008-06-18 04:45:12 [InstanceEdit:384350] Kanapin, AA, 2008-11-26 14:00:39 [InstanceEdit:392885] Kanapin, AA, 2009-03-09 12:07:18 [InstanceEdit:400710] Schmidt, EE, 2009-03-25 05:33:35 [InstanceEdit:423310] Kanapin, AA [InstanceEdit:435478] Kanapin, AA [InstanceEdit:435871] Kanapin, AA [InstanceEdit:447347] Kanapin, AA [InstanceEdit:525883] Kanapin, AA [InstanceEdit:613449] Kanapin, AA [InstanceEdit:797602] Kanapin, AA [InstanceEdit:937368] Yung, CK [InstanceEdit:1042053] Yung, CK [InstanceEdit:1220657] Yung, CK [InstanceEdit:1300696] Yung, CK [InstanceEdit:1301627] Yung, CK [InstanceEdit:1551960] Weiser, JD [InstanceEdit:1995863] Weiser, JD [InstanceEdit:2132304] Weiser, JD [InstanceEdit:2265580] Weiser, JD [InstanceEdit:4341137] Weiser, JD [InstanceEdit:5263031] Weiser, JD [InstanceEdit:5433710] Weiser, JD [InstanceEdit:5618415] Weiser, JD [InstanceEdit:5634237] Weiser, JD [InstanceEdit:5673015] Weiser, JD [InstanceEdit:8944791] Weiser, JD [InstanceEdit:8987656] Weiser, JD [InstanceEdit:9037114] Weiser, JD [InstanceEdit:9637257] Weiser, JD [InstanceEdit:9657908] Weiser, JD [InstanceEdit:9676415] Weiser, JD [InstanceEdit:9715482] Weiser, JD [InstanceEdit:9730071] Weiser, JD [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9917590] Weiser, Joel, 2024-08-09 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 |
| name | SMDT1 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8995406] ENSEMBL:ENSG00000183172 SMDT1 [Homo sapiens] |
| secondaryIdentifier | EMRE_HUMAN B2R5D1 Q8TAB9 |
| sequenceLength | 107 |
| species | [Species:48887] Homo sapiens |
| (referenceEntity) | [EntityWithAccessionedSequence:8949227] SMDT1 [mitochondrial inner membrane] [Homo sapiens] [EntityWithAccessionedSequence:8949642] SMDT1 transit peptide [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:8949655] proSMDT1 [mitochondrial inner membrane] [Homo sapiens] |
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No pathways have been reviewed or authored by UniProt:Q9H4I9 SMDT1 (226174)
