Protein: Calcium uniporter protein, mitochondrial (Q08BI9)
Organism: Danio rerio (Zebrafish) (Brachydanio rerio)
Function: Mitochondrial inner membrane calcium uniporter that mediates calcium uptake into mitochondria. Constitutes the pore-forming and calcium-conducting subunit of the uniporter complex (uniplex). Activity is regulated by micu1 and micu2. At low Ca(2+) levels mcu activity is down-regulated by micu1 and micu2; at higher Ca(2+) levels micu1 increases mcu activity. Mitochondrial calcium homeostasis plays key roles in cellular physiology and regulates cell bioenergetics, cytoplasmic calcium signals and activation of cell death pathways. Involved in buffering the amplitude of systolic calcium rises in cardiomyocytes. While dispensable for baseline homeostatic cardiac function, acts as a key regulator of short-term mitochondrial calcium loading underlying a 'fight-or-flight' response during acute stress: acts by mediating a rapid increase of mitochondrial calcium in pacemaker cells.
Subcellular location: Mitochondrion inner membrane {ECO:0000250|UniProtKB:Q8NE86}; Multi-pass membrane protein {ECO:0000250|UniProtKB:Q8NE86}.
Sequence: MAAKVCRSVLLLSRSSGAVASSAYPAFGVSSQRHQGTKTEALSMSLGGHQTVRRAHGLRTGGRCALFCHPSATLTAQGWKGSPSWQVQRLLCSPAAEDVSVVYQNGLPVISVRLPSRRERCQFTLKPLSDTVGVFLQQLQAEDRGIDRVTIYSADGARIASSTGIDILLMDNFKLVINDTSYLVQPPRRDLLPHEDGERLNDVKILVQQLYTTLRIEEHQLNKERELIGRLEDLNSQLQPLEKVKEELSKKAERRTTWVLWGGMAYMATQFGILARLTWWEYSWDIMEPVTYFITYGTAMAMYAYFVLTRQEYLYPDARDRQYLLFFHRGAKRTRFDIEKYNKLKDAIAEAELDLKRLRDPLQLNLPIQQIDTSKD
InterPro domains: - IPR039055: MCU family (family) [50-361]
- IPR006769: Calcium uniporter protein, C-terminal (domain) [142-344]
Interaction partners: - Calcium uptake protein 1, mitochondrial
- Essential MCU regulator, mitochondrial
- Single-pass membrane protein with aspartate-rich tail 1a.
- Calcium uptake protein 3, mitochondrial isoform X1
- Mitochondrial calcium uptake family, member 3a.
- Mitochondrial calcium uniporter regulator 1
- Mitochondrial proton/calcium exchanger protein/H
- BCL-WAV
- Non-selective voltage-gated ion channel VDAC1
- Calcium-transporting ATPase
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005215 (transporter activity), GO:0022857 (transmembrane transporter activity), GO:1901702 (salt transmembrane transporter activity), GO:0015075 (monoatomic ion transmembrane transporter activity), GO:0022803 (passive transmembrane transporter activity), GO:0015318 (inorganic molecular entity transmembrane transporter activity), GO:0022804 (active transmembrane transporter activity), GO:0005216 (monoatomic ion channel activity), GO:0022890 (inorganic cation transmembrane transporter activity), GO:0015085 (calcium ion transmembrane transporter activity), GO:0015267 (channel activity), GO:0008324 (monoatomic cation transmembrane transporter activity), GO:0015291 (secondary active transmembrane transporter activity), GO:0005261 (monoatomic cation channel activity), GO:0005262 (calcium channel activity), GO:0046873 (metal ion transmembrane transporter activity)
Biological Process (BP): GO:0008150 (biological process), GO:0051179 (localization), GO:0042592 (homeostatic process), GO:0032502 (developmental process), GO:0009987 (cellular process), GO:0032501 (multicellular organismal process), GO:0048878 (chemical homeostasis), GO:0048869 (cellular developmental process), GO:0048856 (anatomical structure development), GO:0019725 (cellular homeostasis), GO:0007275 (multicellular organism development), GO:0055085 (transmembrane transport), GO:0051234 (establishment of localization), GO:0050801 (monoatomic ion homeostasis), GO:0055082 (intracellular chemical homeostasis), GO:0030154 (cell differentiation), GO:0098660 (inorganic ion transmembrane transport), GO:0048731 (system development), GO:0006810 (transport), GO:0048513 (animal organ development), GO:1990542 (mitochondrial transmembrane transport), GO:0098771 (inorganic ion homeostasis), GO:0034220 (monoatomic ion transmembrane transport), GO:0098655 (monoatomic cation transmembrane transport), GO:0006851 (mitochondrial calcium ion transmembrane transport), GO:0098662 (inorganic cation transmembrane transport), GO:0055080 (monoatomic cation homeostasis), GO:0042593 (glucose homeostasis), GO:0006873 (intracellular monoatomic ion homeostasis), GO:0006811 (monoatomic ion transport), GO:0055074 (calcium ion homeostasis), GO:0070588 (calcium ion transmembrane transport), GO:0006812 (monoatomic cation transport), GO:0006874 (intracellular calcium ion homeostasis), GO:0006816 (calcium ion transport), GO:0030003 (intracellular monoatomic cation homeostasis), GO:0030001 (metal ion transport), GO:0051560 (mitochondrial calcium ion homeostasis)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005737 (cytoplasm), GO:0043229 (intracellular organelle), GO:0005739 (mitochondrion), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle)