Biochemical Journal · 2013 · 41 citations · 14 references
Life SciencesBiochemistryCell BiologyCell SignalingIntracellular TransportMitochondrial BiologyMitochondrial BiochemistryMitochondrial BioenergeticsMitochondrial Calcium HomeostasisMitochondrial DynamicsMitochondrial MetabolismMitochondrial PhysiologyCalcium Transport Across MitochondriaGatekeeper AccessMitochondrial Calcium DynamicsMitochondrial Calcium HandlingMitochondrial Calcium Homeostatic MechanismsMitochondrial Calcium Transport ProteinsMitochondrial Calcium Uniporter ComplexMitochondrial Ion Transport
MICU1 (Ca2+ uptake protein 1, mitochondrial) is an important regulator of the MCU (Ca2+ uniporter protein, mitochondrial) that has been shown recently to act as a gatekeeper of the MCU at low [Ca2+]c (cytosolic [Ca2+]). In the present study we have investigated in detail the dynamics of MCU activity after shRNA-knockdown of MICU1 and we have found several new interesting properties. In MICU1-knockdown cells, the rate of mitochondrial Ca2+ uptake was largely increased at a low [Ca2+]c (<2 μM), but it was decreased at a high [Ca2+]c (>4 μM). In the 2-4 μM range a mixed behaviour was observed, where mitochondrial Ca2+ uptake started earlier in the MICU1-silenced cells, but at a lower rate than in the controls. The sensitivity of Ca2+ uptake to Ruthenium Red and Ru360 was similar at both high and low [Ca2+]c, indicating that the same Ca2+ pathway was operating in both cases. The increased Ca2+-uptake rate observed at a [Ca2+]c below 2 μM was transient and became inhibited during Ca2+ entry. Development of this inhibition was slow, requiring 5 min for completion, and was hardly reversible. Therefore MICU1 acts both as a MCU gatekeeper at low [Ca2+]c and as a cofactor necessary to reach the maximum Ca2+-uptake rate at high [Ca2+]c. Moreover, in the absence of MICU1, the MCU becomes sensitive to a slow-developing inhibition that requires prolonged increases in [Ca2+]c in the low micromolar range.
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A forty-kilodalton protein of the inner membrane is the mitochondrial calcium uniporter
Diego De Stefani, Anna Raffaello, Enrico Teardo et al. · Nature · 2011 · 1.9K citations · Full text
MICU1 encodes a mitochondrial EF hand protein required for Ca2+ uptake
Fabiana Perocchi, Vishal M. Gohil, Hany S. Girgis et al. · Nature · 2010 · 876 citations · Full text