Mitochondrial calcium uptake protein 1 (MICU1)

Mitochondrial calcium uptake protein 1 (MICU1) is a single-pass membrane-associated protein located in the mitochondrial intermembrane space, where it serves as a calcium sensor and regulatory subunit for the mitochondrial calcium uniporter (MCU) complex[1][2][3][4][5]. MICU1 contains two EF-hand calcium-binding domains and regulates the MCU channel by setting a threshold for calcium uptake: at low cytosolic calcium, it inhibits MCU, and upon calcium binding (as cytosolic calcium rises), it undergoes conformational changes that relieve inhibition and permit mitochondrial calcium influx[2][3][4][5]. MICU1 also plays a distinct role in maintaining mitochondrial cristae structure and contact site organization (through interactions with the MICOS complex), independently of its calcium transport regulatory activities[7][8][9]. Germline mutations in MICU1 are associated with neuromuscular and metabolic disorders. Currently, MICU1 is considered a key regulatory protein in mitochondrial physiology and cell death, but is not a primary direct drug target[1][5][7][9].

Other names

Calcium uptake protein 1, mitochondrialCBARA1CALCEFHA3Atopy-related autoantigen CALCCalcium-binding atopy-related autoantigen 1MPXPSFLJ12684

Not applicable; MICU1 is a regulatory protein, not currently a direct drug target.