Knowledge (XXG)

Mitochondrial calcium uniporter

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were discovered scientists made some intriguing discoveries in regards to the two proteins. Both MICU1 and MCU share similar RNA sequences, same pattern of expression, and they both interact with one another in the intermitochondrial membrane. It was first found through the use of siRNA screening of the membrane. The functions of MICU1 are still being studied; however, there are some important functions MICU1 plays in the intermitochondrial membrane. MICU1 helps to stabilize the entire mitochondrial calcium uniporter complex, it also limits the amount of calcium that enters the cell during low concentrations of calcium. However, along with limiting the entry of calcium into the mitochondrial matrix, it functions alongside MCU to keep the accumulated calcium inside the matrix of the mitochondria, and promotes ion specificity by preventing aberrant loading of transition metals into the mitochondria.
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sequestered there is reduced calcium; however, whenever MICU1 is sequestered and MICU2 is activated, normal calcium flow. It is also shown that all three, MCU, MICU1, and MICU2 are part of a single complex, the mitochondrial calcium uniporter complex resumes. Research using a CRISPR/Cas9 technique has found that MICU1 and MICU2 play other roles as well. They are essential for cell growth, cell invasion, and cell replication.
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The mitochondrial calcium uptake 1 (MICU1) is a single pass membrane protein, it contains 2 binding domains. This protein was first discovered before the MCU by only a few months. MICU1 was used as a bait to figure out what the core of the mitochondrial calcium uniporter was. Once both MICU1 and MCU
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Mitochondrial calcium uptake 2 (MICU2) is another intermitochondrial membrane protein. It works alongside MICU1 and contains roughly 25% of the same DNA sequence. MICU2 works with MICU1 and MCU to reduce the amount of calcium coming into the matrix. It is shown that when both MICU1 and MICU2 are
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and the concentration of calcium in the cytosol relative to the concentration in the mitochondria. Balancing calcium concentration is necessary to increase the cell's energy supply and regulate cell death. Calcium is balanced through the MCU in conjunction with the
405:) triggers the release of calcium from these intracellular stores, which creates microdomains of high calcium concentration between the ER and the mitochondria, creating the conditions for the MCU to take up calcium. 389:
for calcium, so the cytosolic calcium concentration needs to be approximately 5-10 uM for significant transport of calcium into the mitochondria. Mitochondria are closely associated with the
146: 90: 78: 207: 583:"Oxygen-bridged dinuclear ruthenium amine complex specifically inhibits Ca2+ uptake into mitochondria in vitro and in situ in single cardiac myocytes" 790: 538:
Broekemeier KM, Krebsbach RJ, Pfeiffer DR (October 1994). "Inhibition of the mitochondrial Ca2+ uniporter by pure and impure ruthenium red".
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are typical reagents used to experimentally block the MCU to study its properties and role in mitochondrial signaling.
800: 491:"The mitochondrial calcium uniporter complex: molecular components, structure and physiopathological implications" 379: 154: 386: 398: 224: 805: 390: 340: 289: 150: 731:
Monteith AJ, Miller JM, Beavers WN, Maloney KN, Seifert EL, Hajnoczky G, et al. (December 2021).
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Wettmarshausen J, Goh V, Huang KT, Arduino DM, Tripathi U, Leimpek A, et al. (November 2018).
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The MCU is one of the primary sources of mitochondria uptake of calcium, and flow is dependent on
713: 563: 370: 766: 705: 663: 624:"MICU1 imparts the mitochondrial uniporter with the ability to discriminate between Ca and Mn" 604: 555: 520: 394: 243: 141: 622:
Kamer KJ, Sancak Y, Fomina Y, Meisel JD, Chaudhuri D, Grabarek Z, et al. (August 2018).
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Matlib MA, Zhou Z, Knight S, Ahmed S, Choi KM, Krause-Bauer J, et al. (April 1998).
639: 761: 732: 658: 623: 515: 490: 83: 784: 408: 355: 717: 47: 366:, which together with the MCU make up the mitochondrial calcium uniporter complex. 359: 129: 567: 107: 95: 71: 733:"Mitochondrial calcium uniporter affects neutrophil bactericidal activity during 701: 506: 456:"MCU - Calcium uniporter protein, mitochondrial precursor - Homo sapiens (Human)" 260: 59: 323: 628:
Proceedings of the National Academy of Sciences of the United States of America
267: 599: 582: 648: 770: 709: 667: 524: 393:(ER), at contact sites, which contains stores of cellular calcium ions for 608: 559: 752: 455: 272: 54: 248: 551: 351: 344: 255: 161: 412: 683:"MICU1 Confers Protection from MCU-Dependent Manganese Toxicity" 236: 123: 66: 42: 347: 319: 309: 304: 283: 278: 266: 254: 242: 230: 218: 206: 198: 190: 185: 180: 160: 140: 122: 117: 101: 89: 77: 65: 53: 41: 33: 28: 23: 484: 482: 480: 478: 476: 8: 301: 114: 760: 657: 647: 598: 514: 447: 177: 20: 7: 587:The Journal of Biological Chemistry 540:Molecular and Cellular Biochemistry 14: 489:Marchi S, Pinton P (March 2014). 358:. Its activity is regulated by 337:mitochondrial calcium uniporter 181:Mitochondrial calcium uniporter 24:Mitochondrial calcium uniporter 1: 118:Available protein structures: 791:Genes on human chromosome 10 702:10.1016/j.celrep.2018.10.037 507:10.1113/jphysiol.2013.268235 375:inner mitochondrial membrane 202:C10orf42, CCDC109A, FLJ46135 343:that allows the passage of 16:Group of transport proteins 822: 796:Transmembrane transporters 495:The Journal of Physiology 300: 113: 600:10.1074/jbc.273.17.10223 380:sodium-calcium exchanger 649:10.1073/pnas.1807811115 385:The MCU has a very low 741:Infection and Immunity 735:Staphylococcus aureus 391:endoplasmic reticulum 341:transmembrane protein 753:10.1128/IAI.00551-21 462:. UniProt Consortium 696:(6): 1425–1435.e7. 640:2018PNAS..115E7960K 634:(34): E7960–E7969. 593:(17): 10223–10231. 552:10.1007/bf00944201 399:1,4,5-triphosphate 397:. The presence of 371:membrane potential 801:Calcium signaling 747:(2): IAI0055121. 395:calcium signaling 333: 332: 329: 328: 176: 175: 172: 171: 167:structure summary 813: 775: 774: 764: 728: 722: 721: 687: 678: 672: 671: 661: 651: 619: 613: 612: 602: 578: 572: 571: 535: 529: 528: 518: 486: 471: 470: 468: 467: 452: 302: 178: 115: 21: 821: 820: 816: 815: 814: 812: 811: 810: 781: 780: 779: 778: 730: 729: 725: 685: 680: 679: 675: 621: 620: 616: 580: 579: 575: 537: 536: 532: 488: 487: 474: 465: 463: 454: 453: 449: 444: 435: 426: 421: 419:MICU1 and MICU2 404: 79:OPM superfamily 17: 12: 11: 5: 819: 817: 809: 808: 803: 798: 793: 783: 782: 777: 776: 723: 673: 614: 573: 530: 501:(5): 829–839. 472: 446: 445: 443: 440: 434: 431: 425: 422: 420: 417: 402: 350:from a cell's 331: 330: 327: 326: 321: 317: 316: 311: 307: 306: 298: 297: 287: 281: 280: 276: 275: 270: 264: 263: 258: 252: 251: 246: 240: 239: 234: 228: 227: 222: 216: 215: 210: 204: 203: 200: 196: 195: 192: 188: 187: 183: 182: 174: 173: 170: 169: 164: 158: 157: 144: 138: 137: 127: 120: 119: 111: 110: 105: 99: 98: 93: 87: 86: 81: 75: 74: 69: 63: 62: 57: 51: 50: 45: 39: 38: 35: 31: 30: 26: 25: 15: 13: 10: 9: 6: 4: 3: 2: 818: 807: 804: 802: 799: 797: 794: 792: 789: 788: 786: 772: 768: 763: 758: 754: 750: 746: 742: 738: 736: 727: 724: 719: 715: 711: 707: 703: 699: 695: 691: 684: 677: 674: 669: 665: 660: 655: 650: 645: 641: 637: 633: 629: 625: 618: 615: 610: 606: 601: 596: 592: 588: 584: 577: 574: 569: 565: 561: 557: 553: 549: 545: 541: 534: 531: 526: 522: 517: 512: 508: 504: 500: 496: 492: 485: 483: 481: 479: 477: 473: 461: 457: 451: 448: 441: 439: 432: 430: 423: 418: 416: 414: 410: 409:Ruthenium red 406: 400: 396: 392: 388: 383: 381: 376: 372: 367: 365: 361: 357: 353: 349: 346: 342: 338: 325: 322: 318: 315: 312: 308: 303: 299: 296: 295: 291: 288: 286: 282: 277: 274: 271: 269: 265: 262: 259: 257: 253: 250: 247: 245: 241: 238: 235: 233: 229: 226: 223: 221: 217: 214: 211: 209: 205: 201: 197: 193: 189: 184: 179: 168: 165: 163: 159: 156: 152: 148: 145: 143: 139: 135: 131: 128: 125: 121: 116: 112: 109: 106: 104: 100: 97: 94: 92: 88: 85: 82: 80: 76: 73: 70: 68: 64: 61: 58: 56: 52: 49: 46: 44: 40: 36: 32: 27: 22: 19: 806:Mitochondria 744: 740: 734: 726: 693: 690:Cell Reports 689: 676: 631: 627: 617: 590: 586: 576: 546:(1): 33–40. 543: 539: 533: 498: 494: 464:. Retrieved 459: 450: 436: 427: 407: 384: 368: 356:mitochondria 336: 334: 292: 199:Alt. symbols 18: 460:UniProt.org 339:(MCU) is a 314:Swiss-model 186:Identifiers 91:OPM protein 29:Identifiers 785:Categories 737:infection" 466:2016-02-24 442:References 310:Structures 305:Search for 279:Other data 130:structures 103:Membranome 261:NM_138357 208:NCBI gene 60:IPR018782 771:34871043 718:53209158 710:30403999 668:30082385 525:24366263 387:affinity 324:InterPro 147:RCSB PDB 55:InterPro 762:8853686 659:6112746 636:Bibcode 609:9553073 560:7531818 516:3948548 373:of the 352:cytosol 345:calcium 320:Domains 290:Chr. 10 268:UniProt 48:PF04678 769:  759:  716:  708:  666:  656:  607:  568:516473 566:  558:  523:  513:  273:Q8NE86 256:RefSeq 249:Q8NE86 237:614197 191:Symbol 162:PDBsum 136:  126:  72:1.A.77 34:Symbol 714:S2CID 686:(PDF) 564:S2CID 433:MICU2 424:MICU1 413:Ru360 364:MICU2 360:MICU1 354:into 285:Locus 225:23526 213:90550 767:PMID 706:PMID 664:PMID 605:PMID 556:PMID 521:PMID 411:and 362:and 348:ions 335:The 232:OMIM 220:HGNC 155:PDBj 151:PDBe 134:ECOD 124:Pfam 96:6dnf 67:TCDB 43:Pfam 757:PMC 749:doi 698:doi 654:PMC 644:doi 632:115 595:doi 591:273 548:doi 544:139 511:PMC 503:doi 499:592 401:(IP 294:222 244:PDB 194:MCU 142:PDB 108:216 84:486 37:MCU 787:: 765:. 755:. 745:90 743:. 739:. 712:. 704:. 694:25 692:. 688:. 662:. 652:. 642:. 630:. 626:. 603:. 589:. 585:. 562:. 554:. 542:. 519:. 509:. 497:. 493:. 475:^ 458:. 382:. 153:; 149:; 132:/ 773:. 751:: 720:. 700:: 670:. 646:: 638:: 611:. 597:: 570:. 550:: 527:. 505:: 469:. 403:3

Index

Pfam
PF04678
InterPro
IPR018782
TCDB
1.A.77
OPM superfamily
486
OPM protein
6dnf
Membranome
216
Pfam
structures
ECOD
PDB
RCSB PDB
PDBe
PDBj
PDBsum
structure summary
NCBI gene
90550
HGNC
23526
OMIM
614197
PDB
Q8NE86
RefSeq

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