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
Relevance
Structural highlights
Ca 2+ binds to EF domain on MICU 1 and 2
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