MICU3
entrez
286097
canonical
uniprot
Q86XE3
canonical
ensembl
ENSG00000155970
resolver
ensp
ENSP00000321455
resolver
ensp
ENSP00000427765
resolver
ensp
ENSP00000578192
resolver
ensp
ENSP00000578193
resolver
ensp
ENSP00000590097
resolver
ensp
ENSP00000622746
resolver
ensp
ENSP00000622747
resolver
ensp
ENSP00000622748
resolver
enst
ENST00000318063
resolver
enst
ENST00000519044
resolver
genesymbol
MICU3
resolver
genesymbol_syn
EFHA2
resolver
hgnc
HGNC:27820
resolver
uniprot
H0YAN7
resolver
uniprot
Q8IYZ3
resolver
uniprot_entry
H0YAN7_HUMAN
resolver
uniprot_entry
MICU3_HUMAN
resolver
uniprot_sec
Q8IYZ3
resolver
amino_acid_sequence
MAALRRLLWPPPRVSPPLCAHQPLLGPWGRPAVTTLGLPGRPFSSREDEERAVAEAAWRRRRRWGELSVAAAAGGGLVGLVCYQLYGDPRAGSPATGRPSKSAATEPEDPPRGRGMLPIPVAAAKETVAIGRTDIEDLDLYATSRERRFRLFASIECEGQLFMTPYDFILAVTTDEPKVAKTWKSLSKQELNQMLAETPPVWKGSSKLFRNLKEKGVISYTEYLFLLCILTKPHAGFRIAFNMFDTDGNEMVDKKEFLVLQEIFRKKNEKREIKGDEEKRAMLRLQLYGYHSPTNSVLKTDAEELVSRSYWDTLRRNTSQALFSDLAERADDITSLVTDTTLLVHFFGKKGKAELNFEDFYRFMDNLQTEVLEIEFLSYSNGMNTISEEDFAHILLRYTNVENTSVFLENVRYSIPEEKGITFDEFRSFFQFLNNLEDFAIALNMYNFASRSIGQDEFKRAVYVATGLKFSPHLVNTVFKIFDVDKDDQLSYKEFIGIMKDRLHRGFRGYKTVQKYPTFKSCLKKELHSR
uniprot
proteins
function
FUNCTION: Tissue-specific calcium sensor of the mitochondrial calcium uniporter (MCU) channel, which specifically regulates MCU channel activity in the central nervous system and skeletal muscle (PubMed:29725115). Senses calcium level via its EF-hand domains: compared to MICU1 and MICU2, MICU3 has a higher affinity for calcium (PubMed:29725115). MICU1 and MICU3 form a disulfide-linked heterodimer that stimulates and inhibits MCU activity, depending on the concentration of calcium (PubMed:29725115). At low calcium levels, MICU1 occludes the pore of the MCU channel, preventing mitochondrial calcium uptake (PubMed:29725115). At higher calcium levels, calcium-binding to MICU1 and MICU3 induces a conformational change that weakens MCU-MICU1 interactions and moves the MICU1-MICU3 heterodimer away from the pore, allowing calcium permeation through the MCU channel (PubMed:29725115). The high calcium affinity of MICU3 lowers the calcium threshold necessary for calcium permeation through the MCU channel (PubMed:29725115). The MICU1-MICU3 heterodimer promotes flexibility of neurotransmission in neuronal cells by enhancing mitochondrial calcium uptake in presynapses (PubMed:29725115). It is also required to increase mitochondrial calcium uptake in skeletal muscle cells, thereby increasing ATP production (By similarity). {ECO:0000250|UniProtKB:Q9CTY5, ECO:0000269|PubMed:29725115}.
uniprot
proteins
mass_dalton
60711
uniprot
proteins
mutagenesis
MUTAGEN 245; /note="D->A: In EF1(mut); abolished calcium-binding; when associated with K-256, A-483 and K-494."; /evidence="ECO:0000269|PubMed:29725115"; MUTAGEN 256; /note="E->K: In EF1(mut); abolished calcium-binding; when associated with A-245, A-483 and K-494."; /evidence="ECO:0000269|PubMed:29725115"; MUTAGEN 483; /note="D->A: In EF1(mut); abolished calcium-binding; when associated with A-245, K-256 and K-494."; /evidence="ECO:0000269|PubMed:29725115"; MUTAGEN 494; /note="E->K: In EF1(mut); abolished calcium-binding; when associated with A-245, K-256 and A-483."; /evidence="ECO:0000269|PubMed:29725115"; MUTAGEN 522; /note="C->A: Abolished interaction with MICU1."; /evidence="ECO:0000269|PubMed:29725115
uniprot
proteins
protein_family
MICU1 family
uniprot
proteins
protein_family
MICU3 subfamily
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16421571
uniprot
proteins
pubmed
29725115
uniprot
proteins
pubmed
30699349
uniprot
proteins
sequence_length
530
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion intermembrane space {ECO:0000250|UniProtKB:Q8IYU8}. Mitochondrion inner membrane {ECO:0000250|UniProtKB:Q8IYU8}. Note=Recruited to the mitochondrial inner membrane via its association with the uniplex complex. {ECO:0000250|UniProtKB:Q8IYU8}.
uniprot
proteins