Calcium uptake protein 1

Structure)

spinqualitylabelsall models Caption for this structure Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Micu 1 (Mitochondrial Calcium Uptake 1) is a key regulator of mitochondrial calcium uniporter (MCU) required to increase calcium uptake by MCU when cytoplasmic calcium is high. It also regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake. Function Micu 1 is a regulator of Ca 2+ uptakes in mitochondria. It mainly acts on MCU when linked to MICU2 (paralogues). When in an hexameric conformation : Micu 1 silences the activity of MCU/EMRE and so stop the Ca 2+ from entering the mitochondria. When the calcium of the intermembrane binds to MICU1 and MICU2 they change their conformation and open the “gate” in front of the MCU channel and let the calcium enter into the matrix. Disease The major disease linked to a dysfunctional Micu 1 protein is myopathy with extrapyramidal signs (MPXPS). But other diseases can be observed like : ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy Structural highlights Ca 2+ binds to EF domain on MICU 1 and 2 Here is the binding site of calcium in the dimer This is a sample scene created with SAT to color by Group, and another to make a transparent representation of the protein. You can make your own scenes on SAT starting from scratch or loading and editing one of these sample scenes.

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Alexandra Helleux, Michal Harel, Jaime Prilusky