The mitochondrial calcium uniporter complex: Molecular components, structure and physiopathological implications

Journal of Physiology

Volumn 592, Issue 5, 2014, Pages 829-839

  Marchi, Saverio ^a   Pinton, Paolo ^a  

^a UNIVERSITY OF FERRARA   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

CALCIUM ION; MITOCHONDRIAL CALCIUM UNIPORTER; MITOCHONDRIAL CALCIUM UNIPORTER COMPLEX; MITOCHONDRIAL CALCIUM UPTAKE 1 PROTEIN; MITOCHONDRIAL CALCIUM UPTAKE 2 PROTEIN; MITOCHONDRIAL PROTEIN; PROTEIN EMRE; PROTEIN MCUB; PROTEIN MCUR1; PROTEIN MIR 25; REGULATOR PROTEIN; UNCLASSIFIED DRUG;

AMINO ACID SEQUENCE; CALCIUM CELL LEVEL; CALCIUM CONDUCTANCE; CALCIUM STONE; CALCIUM TRANSPORT; HUMAN; MITOCHONDRIAL MEMBRANE; MITOCHONDRION; MOLECULAR BIOLOGY; MOLECULAR DYNAMICS; NONHUMAN; PATHOPHYSIOLOGY; PRIORITY JOURNAL; PROTEIN DOMAIN; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN MOTIF; PROTEIN PROTEIN INTERACTION; PROTEIN STRUCTURE; REVIEW;

ANIMALS; CALCIUM; CALCIUM CHANNELS; CALCIUM SIGNALING; HUMANS; MEMBRANE POTENTIAL, MITOCHONDRIAL; MITOCHONDRIA; MITOCHONDRIAL DISEASES; MODELS, BIOLOGICAL; MODELS, CHEMICAL; STRUCTURE-ACTIVITY RELATIONSHIP;

EID: 84896718004     PISSN: 00223751     EISSN: 14697793     Source Type: Journal    
DOI: 10.1113/jphysiol.2013.268235     Document Type: Review

References (59)

1. 2+ uniporter (MCU) contribute to metabolism-secretion coupling in clonal pancreatic beta-cells. J Biol Chem 287, 34445-34454.
2. Altschuld RA, Hohl CM, Castillo LC, Garleb AA, Starling RC & Brierley GP (1992). Cyclosporin inhibits mitochondrial calcium efflux in isolated adult rat ventricular cardiomyocytes. Am J Physiol Heart Circ Physiol 262, H1699-H1704.
3. Baughman JM, Perocchi F, Girgis HS, Plovanich M, Belcher-Timme CA, Sancak Y, Bao XR, Strittmatter L, Goldberger O, Bogorad RL, Koteliansky V & Mootha VK (2011). Integrative genomics identifies MCU as an essential component of the mitochondrial calcium uniporter. Nature 476, 341-345.
4. Bick AG, Calvo SE & Mootha VK (2012). Evolutionary diversity of the mitochondrial calcium uniporter. Science 336, 886.
5. Bonora M, Giorgi C, Bononi A, Marchi S, Patergnani S, Rimessi A, Rizzuto R & Pinton P (2013). Subcellular calcium measurements in mammalian cells using jellyfish photoprotein aequorin-based probes. Nat Protoc 8, 2105-2118.
6. Bonora M, Patergnani S, Rimessi A, De Marchi E, Suski JM, Bononi A, Giorgi C, Marchi S, Missiroli S, Poletti F, Wieckowski MR & Pinton P (2012). ATP synthesis and storage. Purinergic Signal 8, 343-357.
7. 2+ carrier in rat liver mitochondria. Biochemistry 18, 5972-5978.
8. 2+ from liver mitochondria. Biochem J 225, 413-419.
9. 2+ metabolism in yeast cells and mitochondria. Biochim Biophys Acta 205, 18-26.
10. 2+ transfer to mitochondria. Cell 142, 270-283.
11. Chaudhuri D, Sancak Y, Mootha VK & Clapham DE (2013). MCU encodes the pore conducting mitochondrial calcium currents. Elife 2, e00704.
12. 2+ uniporter. Cell Metab 17, 976-987.
13. 2+-affinity targeted aequorin. Cell Calcium 54, 37-45.
14. De Stefani D, Raffaello A, Teardo E, Szabo I & Rizzuto R (2011). A forty-kilodalton protein of the inner membrane is the mitochondrial calcium uniporter. Nature 476, 336-340.
15. Dimmer KS, Navoni F, Casarin A, Trevisson E, Endele S, Winterpacht A, Salviati L & Scorrano L (2008). LETM1, deleted in Wolf-Hirschhorn syndrome is required for normal mitochondrial morphology and cellular viability. Hum Mol Genet 17, 201-214.
16. Docampo R & Lukes J (2012). Trypanosomes and the solution to a 50-year mitochondrial calcium mystery. Trends Parasitol 28, 31-37.
17. 2+ peaks in cardiomyocytes. Proc Natl Acad Sci U S A 109, 12986-12991.
18. Drago I, Pizzo P & Pozzan T (2011). After half a century mitochondrial calcium in- and efflux machineries reveal themselves. EMBO J 30, 4119-4125.
19. Feng S, Li H, Tai Y, Huang J, Su Y, Abramowitz J, Zhu MX, Birnbaumer L & Wang Y (2013). Canonical transient receptor potential 3 channels regulate mitochondrial calcium uptake. Proc Natl Acad Sci U S A 110, 11011-11016.
20. Fieni F, Lee SB, Jan YN & Kirichok Y (2012). Activity of the mitochondrial calcium uniporter varies greatly between tissues. Nat Commun 3, 1317.
21. 2+ and apoptosis. Cell Calcium 52, 36-43.
22. Hoffman NE, Chandramoorthy HC, Shamugapriya S, Zhang X, Rajan S, Mallilankaraman K, Gandhirajan RK, Vagnozzi RJ, Ferrer LM, Sreekrishnanilayam K, Natarajaseenivasan K, Vallem S, Force T, Choi ET, Cheung JY & Madesh M (2013). MICU1 Motifs Define Mitochondrial Calcium Uniporter Binding and Activity. Cell Rep.
23. Huang G, Vercesi AE & Docampo R (2013). Essential regulation of cell bioenergetics in Trypanosoma brucei by the mitochondrial calcium uniporter. Nat Commun 4, 2865.
24. Igbavboa U & Pfeiffer DR (1991). Regulation of reverse uniport activity in mitochondria by extramitochondrial divalent cations. Dependence on a soluble intermembrane space component. J Biol Chem 266, 4283-4287.
25. + antiporter. Science 326, 144-147.
26. Joiner ML, Koval OM, Li J, He BJ, Allamargot C, Gao Z, Luczak ED, Hall DD, Fink BD, Chen B, Yang J, Moore SA, Scholz TD, Strack S, Mohler PJ, Sivitz WI, Song LS & Anderson ME (2012). CaMKII determines mitochondrial stress responses in heart. Nature 491, 269-273.
27. Kirichok Y, Krapivinsky G & Clapham DE (2004). The mitochondrial calcium uniporter is a highly selective ion channel. Nature 427, 360-364.
28. 2+ uptake that regulates cellular metabolism. Nat Cell Biol 14, 1336-1343.
29. 2+ uptake that regulates cell survival. Cell 151, 630-644.
30. Marchi S, Lupini L, Patergnani S, Rimessi A, Missiroli S, Bonora M, Bononi A, Corra F, Giorgi C, De Marchi E, Poletti F, Gafa R, Lanza G, Negrini M, Rizzuto R & Pinton P (2013). Downregulation of the mitochondrial calcium uniporter by cancer-related miR-25. Curr Biol 23, 58-63.
31. Martell JD, Deerinck TJ, Sancak Y, Poulos TL, Mootha VK, Sosinsky GE, Ellisman MH & Ting AY (2012). Engineered ascorbate peroxidase as a genetically encoded reporter for electron microscopy. Nat Biotechnol 30, 1143-1148.
32. Nicholls DG & Crompton M (1980). Mitochondrial calcium transport. FEBS Lett 111, 261-268.
33. Pagliarini DJ, Calvo SE, Chang B, Sheth SA, Vafai SB, Ong SE, Walford GA, Sugiana C, Boneh A, Chen WK, Hill DE, Vidal M, Evans JG, Thorburn DR, Carr SA & Mootha VK (2008). A mitochondrial protein compendium elucidates complex I disease biology. Cell 134, 112-123.
34. 2+ exchange. Proc Natl Acad Sci U S A 107, 436-441.
35. Pan X, Liu J, Nguyen T, Liu C, Sun J, Teng Y, Fergusson MM, Rovira, II, Allen M, Springer DA, Aponte AM, Gucek M, Balaban RS, Murphy E & Finkel T (2013). The physiological role of mitochondrial calcium revealed by mice lacking the mitochondrial calcium uniporter. Nat Cell Biol 15, 1464-1472.
36. 2+-binding glycoprotein. Nature 264, 185-186.
37. Patergnani S, Suski JM, Agnoletto C, Bononi A, Bonora M, De Marchi E, Giorgi C, Marchi S, Missiroli S, Poletti F, Rimessi A, Duszynski J, Wieckowski MR & Pinton P (2011). Calcium signalling around Mitochondria Associated Membranes (MAMs). Cell Commun Signal 9, 19.
38. 2+ uptake. Nature 467, 291-296.
39. Plovanich M, Bogorad RL, Sancak Y, Kamer KJ, Strittmatter L, Li AA, Girgis HS, Kuchimanchi S, De Groot J, Speciner L, Taneja N, Oshea J, Koteliansky V & Mootha VK (2013). MICU2, a paralog of MICU1, resides within the mitochondrial uniporter complex to regulate calcium handling. PLoS One 8, e55785.
40. Prudent J, Popgeorgiev N, Bonneau B, Thibaut J, Gadet R, Lopez J, Gonzalo P, Rimokh R, Manon S, Houart C, Herbomel P, Aouacheria A & Gillet G (2013). Bcl-wav and the mitochondrial calcium uniporter drive gastrula morphogenesis in zebrafish. Nat Commun 4, 2330.
41. Qiu J, Tan YW, Hagenston AM, Martel MA, Kneisel N, Skehel PA, Wyllie DJ, Bading H & Hardingham GE (2013). Mitochondrial calcium uniporter Mcu controls excitotoxicity and is transcriptionally repressed by neuroprotective nuclear calcium signals. Nat Commun 4, 2034.
42. Raffaello A, De Stefani D, Sabbadin D, Teardo E, Merli G, Picard A, Checchetto V, Moro S, Szabo I & Rizzuto R (2013). The mitochondrial calcium uniporter is a multimer that can include a dominant-negative pore-forming subunit. EMBO J 32, 2362-2376.
43. Rhee HW, Zou P, Udeshi ND, Martell JD, Mootha VK, Carr SA & Ting AY (2013). Proteomic mapping of mitochondria in living cells via spatially restricted enzymatic tagging. Science 339, 1328-1331.
44. Rizzuto R, De Stefani D, Raffaello A & Mammucari C (2012). Mitochondria as sensors and regulators of calcium signalling. Nat Rev Mol Cell Biol 13, 566-578.
45. 2+ responses. Science 280, 1763-1766.
46. Rudolf R, Mongillo M, Rizzuto R & Pozzan T (2003). Looking forward to seeing calcium. Nat Rev Mol Cell Biol 4, 579-586.
47. Sancak Y, Markhard AL, Kitami T, Kovacs-Bogdan E, Kamer KJ, Udeshi ND, Carr SA, Chaudhuri D, Clapham DE, Li AA, Calvo SE, Goldberger O & Mootha VK (2013). EMRE is an essential component of the mitochondrial calcium uniporter complex. Science 342, 1379-1382.
48. Santo-Domingo J & Demaurex N (2010). Calcium uptake mechanisms of mitochondria. Biochim Biophys Acta 1797, 907-912.
49. Saris NE, Sirota TV, Virtanen I, Niva K, Penttila T, Dolgachova LP & Mironova GD (1993). Inhibition of the mitochondrial calcium uniporter by antibodies against a 40-kDa glycoproteinT. J Bioenerg Biomembr 25, 307-312.
50. Somlyo AP (1984). Cell physiology: cellular site of calcium regulation. Nature 309, 516-517.
51. Somlyo AP, Bond M & Somlyo AV (1985). Calcium content of mitochondria and endoplasmic reticulum in liver frozen rapidly in vivo. Nature 314, 622-625.
52. 2+ binding glycoprotein from ox liver mitochondria. Biochem Biophys Res Commun 47, 808-813.
53. Sparagna GC, Gunter KK, Sheu SS & Gunter TE (1995). Mitochondrial calcium uptake from physiological-type pulses of calcium. A description of the rapid uptake mode. J Biol Chem 270, 27510-27515.
54. 2+ from a nonmitochondrial intracellular store in pancreatic acinar cells by inositol-1, 4, 5-trisphosphate. Nature 306, 67-69.
55. 2+ accumulation regulates ATP synthesis in pancreatic beta cells. Pflugers Arch 465, 543-554.
56. 2+ uniporter MCU is essential for glucose-induced ATP increases in pancreatic beta-cells. PLoS One 7, e39722.
57. 2+ uniport. Nat Cell Biol 9, 445-452.
58. 2+ influx and efflux rates in guinea pig cardiac mitochondria: low and high affinity effects of cyclosporine A. Biochim Biophys Acta 1813, 1373-1381.
59. Zazueta C, Holguin JA & Ramirez J (1991). Calcium transport sensitive to ruthenium red in cytochrome oxidase vesicles reconstituted with mitochondrial proteins. J Bioenerg Biomembr 23, 889-902.