Knowledge (XXG)

Prekallikrein

Source đź“ť

126:
PK was initially described by Hathaway et al. in 1965 after encountering a Kentucky family who exhibited strikingly abnormal APTT results, but showed no bleeding symptoms. The family appeared to have a hereditary deficiency in an unknown coagulation factor, dubbed “Fletcher factor” after the family.
117:
Although most cases of prekallikrein deficiency are asymptomatic, a few reports link severe prekallikrein deficiency with thrombotic phenomena and recurrent pregnancy loss. More recently, a case of prekallikrein deficiency was shown to be associated with severe mucosal bleeding.
76:
domain. The four apple domains create a disk-like platform around the base of the catalytic domain. However, unlike factor XI, prekallikrein does not form dimers.
281: 673: 107: 758: 542: 174:"Molecular modeling of the prekallikrein structure provides insights into high-molecular-weight kininogen binding and zymogen activation" 247: 274: 156: 668: 590: 585: 719: 213:
Dasanu CA, Alexandrescu DT (November 2009). "A case of prekallikrein deficiency resulting in severe recurrent mucosal hemorrhage".
547: 383: 31: 323: 623: 267: 575: 753: 663: 39: 489: 485: 604: 348: 317: 645: 655: 632: 613: 595: 290: 111: 298: 61: 683: 640: 230: 195: 152: 87:
cleavage of a bond homologous to the corresponding bond cleaved during factor XI activation.
696: 691: 222: 185: 363: 342: 328: 73: 27: 701: 127:
In 1973 Kirk Wuepper determined that Fletcher factor and prekallikrein were the same.
747: 514: 438: 190: 173: 711: 557: 522: 226: 565: 500: 471: 453: 428: 418: 373: 307: 481: 443: 403: 398: 388: 103: 84: 80: 69: 43: 35: 580: 537: 532: 527: 413: 408: 102:
Deficiencies in PK can also be acquired due to some disease states, such as
65: 55: 234: 199: 476: 466: 461: 95:
Hereditary deficiencies in PK are very rare. They can cause a prolonged
728: 570: 259: 495: 352: 337: 332: 357: 251: 99:, which can be corrected by incubation of the patient’s plasma. 96: 263: 543:
Protein Z-related protease inhibitor (ZPI) (inhibits FX, FXI)
149:
Disorders of Hemostasis & Thrombosis: A Clinical Guide
548:
Tissue factor pathway inhibitor (TFPI) (inhibits FIII)
710: 682: 654: 631: 622: 556: 513: 452: 427: 372: 306: 297: 42:. PK is cleaved to produce kallikrein by activated 614:Thrombin-activatable fibrinolysis inhibitor (TAFI) 674:Activated protein C–protein C inhibitor (APC–PCI) 523:Antithrombin (inhibits FII, FIX, FX, FXI, FXII) 275: 8: 38:, which is a serine protease that activates 142: 140: 628: 303: 282: 268: 260: 172:Hooley E, McEwan PA, Emsley J (Dec 2007). 151:(2nd ed.). McGraw Hill Professional. 591:Plasminogen activator inhibitor-2 (PAI-2) 586:Plasminogen activator inhibitor-1 (PAI-1) 189: 147:Goodnight, S.H.; Hathaway, W.E. (2001). 252:KALLIKREIN B, PLASMA, 1; KLKB1 - 229000 136: 384:High-molecular-weight kininogen (HMWK) 178:Journal of Thrombosis and Haemostasis 7: 669:Thrombin–antithrombin complex (TAT) 248:Online Mendelian Inheritance in Man 79:Prekallikrein is activated to form 576:Tissue plasminogen activator (tPA) 533:Protein S (cofactor for protein C) 14: 68:, and similarly consists of four 191:10.1111/j.1538-7836.2007.02792.x 34:. PK is the precursor of plasma 664:Prothrombin fragment 1+2 (F1+2) 324:Platelet membrane glycoproteins 32:high-molecular-weight kininogen 528:Protein C (inhibits FV, FVIII) 1: 227:10.1097/MAJ.0b013e3181b270bb 404:Factor XII (Hageman factor) 318:von Willebrand factor (vWF) 775: 724:-antiplasmin complex (PAP) 439:Factor III (tissue factor) 122:Discovery of prekallikrein 56:Factor_XI § Structure 53: 646:β-Thromboglobulin (β-TG) 91:Prekallikrein deficiency 759:Kinin–kallikrein system 641:Platelet factor 4 (PF4) 538:Protein Z (inhibits FX) 472:Prothrombin (factor II) 72:and a fifth, catalytic 697:Fibrinopeptide B (FpB) 692:Fibrinopeptide A (FpA) 364:Glycoprotein VI (GPVI) 329:Glycoprotein Ib (GPIb) 515:Anticoagulant factors 482:Fibrinogen (factor I) 477:Thrombin (factor IIa) 349:Glycoprotein IIb/IIIa 343:Glycoprotein IX (GP9) 310:(platelet activation) 702:Fibrin monomers (FM) 558:Fibrinolytic factors 376:(contact activation) 30:that complexes with 22:(PK), also known as 656:Thrombin generation 633:Platelet activation 624:Coagulation markers 299:Coagulation factors 291:Coagulation cascade 112:sickle-cell disease 754:Coagulation system 496:Fibrin (factor Ia) 308:Primary hemostasis 46:(Hageman factor). 26:, is an 85,000 Mr 741: 740: 737: 736: 684:Fibrin generation 509: 508: 429:Extrinsic pathway 374:Intrinsic pathway 60:Prekallikrein is 766: 629: 304: 284: 277: 270: 261: 254: 245: 239: 238: 210: 204: 203: 193: 169: 163: 162: 144: 774: 773: 769: 768: 767: 765: 764: 763: 744: 743: 742: 733: 723: 706: 678: 650: 618: 608: 599: 552: 505: 448: 431:(tissue factor) 430: 423: 375: 368: 309: 293: 288: 258: 257: 246: 242: 215:Am. J. Med. Sci 212: 211: 207: 171: 170: 166: 159: 146: 145: 138: 133: 124: 93: 74:serine protease 58: 52: 28:serine protease 24:Fletcher factor 17: 16:Serine protease 12: 11: 5: 772: 770: 762: 761: 756: 746: 745: 739: 738: 735: 734: 732: 731: 726: 721: 716: 714: 708: 707: 705: 704: 699: 694: 688: 686: 680: 679: 677: 676: 671: 666: 660: 658: 652: 651: 649: 648: 643: 637: 635: 626: 620: 619: 617: 616: 611: 609:-Macroglobulin 606: 602: 597: 593: 588: 583: 578: 573: 568: 562: 560: 554: 553: 551: 550: 545: 540: 535: 530: 525: 519: 517: 511: 510: 507: 506: 504: 503: 498: 493: 479: 474: 469: 464: 458: 456: 454:Common pathway 450: 449: 447: 446: 441: 435: 433: 425: 424: 422: 421: 416: 411: 406: 401: 396: 391: 386: 380: 378: 370: 369: 367: 366: 361: 355: 346: 340: 335: 320: 314: 312: 301: 295: 294: 289: 287: 286: 279: 272: 264: 256: 255: 240: 205: 184:(12): 2461–6. 164: 158:978-0071348348 157: 135: 134: 132: 129: 123: 120: 92: 89: 51: 48: 15: 13: 10: 9: 6: 4: 3: 2: 771: 760: 757: 755: 752: 751: 749: 730: 727: 725: 718: 717: 715: 713: 709: 703: 700: 698: 695: 693: 690: 689: 687: 685: 681: 675: 672: 670: 667: 665: 662: 661: 659: 657: 653: 647: 644: 642: 639: 638: 636: 634: 630: 627: 625: 621: 615: 612: 610: 603: 601: 594: 592: 589: 587: 584: 582: 579: 577: 574: 572: 569: 567: 564: 563: 561: 559: 555: 549: 546: 544: 541: 539: 536: 534: 531: 529: 526: 524: 521: 520: 518: 516: 512: 502: 499: 497: 494: 491: 487: 483: 480: 478: 475: 473: 470: 468: 465: 463: 460: 459: 457: 455: 451: 445: 442: 440: 437: 436: 434: 432: 426: 420: 417: 415: 412: 410: 407: 405: 402: 400: 397: 395: 394:Prekallikrein 392: 390: 387: 385: 382: 381: 379: 377: 371: 365: 362: 359: 356: 354: 350: 347: 344: 341: 339: 336: 334: 330: 327: 325: 321: 319: 316: 315: 313: 311: 305: 302: 300: 296: 292: 285: 280: 278: 273: 271: 266: 265: 262: 253: 249: 244: 241: 236: 232: 228: 224: 221:(5): 429–30. 220: 216: 209: 206: 201: 197: 192: 187: 183: 179: 175: 168: 165: 160: 154: 150: 143: 141: 137: 130: 128: 121: 119: 115: 113: 109: 106:, infection, 105: 100: 98: 90: 88: 86: 82: 77: 75: 71: 70:apple domains 67: 63: 57: 49: 47: 45: 41: 37: 33: 29: 25: 21: 20:Prekallikrein 729:D-Dimer (DD) 712:Fibrinolysis 600:-Antiplasmin 393: 322: 243: 218: 214: 208: 181: 177: 167: 148: 125: 116: 101: 94: 78: 59: 23: 19: 18: 566:Plasminogen 501:Factor XIII 419:Factor VIII 748:Categories 444:Factor VII 399:Kallikrein 389:Bradykinin 131:References 104:angioedema 85:factor XII 81:kallikrein 62:homologous 54:See also: 44:Factor XII 36:kallikrein 720:Plasmin-α 581:Urokinase 414:Factor IX 409:Factor XI 66:factor XI 50:Structure 467:Factor V 462:Factor X 250:(OMIM): 235:19773642 200:17922805 571:Plasmin 358:GPIIIa 233:  198:  155:  110:, and 40:kinins 353:GPIIb 338:GP1BB 333:GP1BA 231:PMID 196:PMID 153:ISBN 97:APTT 490:FGG 486:FGA 223:doi 219:338 186:doi 108:DIC 83:by 64:to 750:: 488:, 229:. 217:. 194:. 180:. 176:. 139:^ 114:. 722:2 607:2 605:α 598:2 596:α 492:) 484:( 360:) 351:( 345:) 331:( 326:: 283:e 276:t 269:v 237:. 225:: 202:. 188:: 182:5 161:.

Index

serine protease
high-molecular-weight kininogen
kallikrein
kinins
Factor XII
Factor_XI § Structure
homologous
factor XI
apple domains
serine protease
kallikrein
factor XII
APTT
angioedema
DIC
sickle-cell disease


ISBN
978-0071348348
"Molecular modeling of the prekallikrein structure provides insights into high-molecular-weight kininogen binding and zymogen activation"
doi
10.1111/j.1538-7836.2007.02792.x
PMID
17922805
doi
10.1097/MAJ.0b013e3181b270bb
PMID
19773642
Online Mendelian Inheritance in Man

Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.

↑