Single-pass membrane protein with aspartate-rich tail 1 (essential MCU regulator) (SMDT1)

SMDT1 (Single-pass membrane protein with aspartate-rich tail 1; commonly known as EMRE) is the essential regulatory subunit of the mitochondrial calcium uniporter (MCU) complex, a multi-protein channel required for calcium uptake into the mitochondrial matrix[1][2][3][4]. EMRE/SMDT1 is a small (~11 kDa), single-pass transmembrane protein localized to the inner mitochondrial membrane. Its structure includes an acidic tail exposed to the mitochondrial matrix, acting as a calcium sensor[2]. EMRE is indispensable in linking the pore-forming MCU to its regulatory calcium-sensing proteins (MICU1, MICU2), ensuring tight regulation of channel activity in response to calcium signaling[1][2][3][4]. EMRE stabilizes the MCU complex, acts as a molecular scaffold, and maintains the channel in the open state under appropriate conditions[3][4]. Pathogenic mutations or imbalances in EMRE expression have been associated with disorders of mitochondrial function and calcium homeostasis, including neurological disease[1][2]. There are no known therapeutic agents or biomarkers that specifically target SMDT1/EMRE, but its central regulatory role establishes it as a potential target in diseases involving mitochondrial calcium dysregulation.

Other names

EMREEssential MCU regulator, mitochondrialC22orf32DDDDUPF0466 protein C22orf32dJ186O1.1essential MCU regulator, mitochondrialsingle-pass membrane protein with aspartate-rich tail 1, mitochondrial