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CBS domain

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563:​) manner forming a disk-like compact structure. By doing so, they form clefts that constitute the canonical ligand binding regions. In principle, the number of canonical binding sites matches the number of CBS domains within the molecule and are traditionally numbered according to the CBS domain that contains each of the conserved aspartate residues that potentially interact with the ribose of the nucleotides. However, not all of these cavities might necessarily bind nucleotides or be functional. Recently, a non-canonical site for AMP has also been described in protein MJ1225 from 594:, or s-adenosylmethionine, but they may also bind metallic ions such as Mg. Upon binding these different ligands the CBS domains regulate the activity of associated enzymatic domains. The molecular mechanisms underlying this regulation are just starting to be elucidated. At the moment, two different type of mechanisms have been proposed. The first one claims that the nucleotide portion of the ligand induces essentially no change in the protein structure, the 631: 571: 26: 1786:
Tuominen H, Salminen A, Oksanen E, Jämsen J, Heikkilä O, Lehtiö L, Magretova NN, Goldman A, Baykov AA, Lahti R (May 2010). "Crystal structures of the CBS and DRTGG domains of the regulatory region of Clostridiumperfringens pyrophosphatase complexed with the inhibitor, AMP, and activator, diadenosine
1618:
Lucas M, Encinar JA, Arribas EA, Oyenarte I, García IG, Kortazar D, Fernández JA, Mato JM, Martínez-Chantar ML, Martínez-Cruz LA (February 2010). "Binding of S-methyl-5'-thioadenosine and S-adenosyl-L-methionine to protein MJ0100 triggers an open-to-closed conformational change in its CBS motif
627:, membrane transporters or DNA-binding domains. However, proteins that contain only CBS domains are also often found, particularly in prokaryotes. These standalone CBS domain proteins might form complexes upon binding to other proteins such as kinases to which they interact with and regulate. 598:
at the binding site being the most significant property of adenosine nucleotide binding. This "static" response would be involved in processes in which regulation by energy charge would be advantageous. On the contrary, the second type of mechanism (denoted as "dynamic") involves dramatic
1918:
Tuominen H, Salminen A, Oksanen E, et al. (May 2010). "Crystal Structures of the CBS and DRTGG Domains of the Regulatory Region of Clostridium perfringens Pyrophosphatase Complexed with the Inhibitor, AMP, and Activator, Diadenosine Tetraphosphate".
1825:
Xiao B, Heath R, Saiu P, Leiper FC, Leone P, Jing C, Walker PA, Haire L, Eccleston JF, Davis CT, Martin SR, Carling D, Gamblin SJ (September 2007). "Structural basis for AMP binding to mammalian AMP-activated protein kinase".
1577:
GĂłmez-GarcĂ­a I, Oyenarte I, MartĂ­nez-Cruz LA (May 2010). "The Crystal Structure of Protein MJ1225 from Methanocaldococcus jannaschii Shows Strong Conservation of Key Structural Features Seen in the Eukaryal gamma-AMPK".
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on one side. CBS domains are always found in pairs in protein sequences and each pair of these domains tightly associate in a pseudo dimeric arrangement through their β-sheets forming a so-called CBS-pair or
1264:
Zhang R, Evans G, Rotella FJ, Westbrook EM, Beno D, Huberman E, Joachimiak A, Collart FR (April 1999). "Characteristics and crystal structure of bacterial inosine-5'-monophosphate dehydrogenase".
161: 1087:
Day P, Sharff A, Parra L, et al. (May 2007). "Structure of a CBS-domain pair from the regulatory gamma1 subunit of human AMPK in complex with AMP and ZMP".
488:(AMPK). CBS domains regulate the activity of associated enzymatic and transporter domains in response to binding molecules with adenosyl groups such as 1350:
Meyer S, Savaresi S, Forster IC, Dutzler R (January 2007). "Nucleotide recognition by the cytoplasmic domain of the human chloride transporter ClC-5".
1044:
Meyer S, Savaresi S, Forster IC, Dutzler R (January 2007). "Nucleotide recognition by the cytoplasmic domain of the human chloride transporter ClC-5".
73: 599:
conformational changes in the protein structure upon ligand binding and has been reported for the cytosolic domain of the Mg transporter MgtE from
1871:"Purification, crystallization and preliminary X-ray diffraction analysis of the CBS-domain pair from the Methanococcus jannaschii protein MJ0100" 660: 1396:
Amodeo GA, Rudolph MJ, Tong L (September 2007). "Crystal structure of the heterotrimer core of Saccharomyces cerevisiae AMPK homologue SNF1".
97: 30:
Structure of the yeast SNF4 protein that contains four CBS domains. This protein is part of the AMP-activated protein kinase (AMPK) complex.
477: 956:"Removal of gating in voltage-dependent ClC-2 chloride channel by point mutations affecting the pore and C-terminus CBS-2 domain" 2038:"Albers-Schönberg disease (autosomal dominant osteopetrosis, type II) results from mutations in the ClCN7 chloride channel gene" 682:
genes, and mutations in the CBS domains of several of these have been identified as the cause of genetic diseases. Mutations in
1956:"Mutations in the regulatory domain of cystathionine beta synthase can functionally suppress patient-derived mutations in cis" 886:
Ponting CP (March 1997). "CBS domains in CIC chloride channels implicated in myotonia and nephrolithiasis (kidney stones)".
181: 485: 809:
Bateman A (January 1997). "The structure of a domain common to archaebacteria and the homocystinuria disease protein".
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of CBS domains showing secondary structures above. Yellow arrows represent beta strands and red boxes alpha helices.
1005:"The structure of the cytoplasmic domain of the chloride channel ClC-Ka reveals a conserved interaction interface" 117: 643:
Mutations in some human CBS domain-containing proteins leads to genetic diseases. For example, mutations in the
2082: 1739:"CBS domains form energy-sensing modules whose binding of adenosine ligands is disrupted by disease mutations" 169: 476:, one of the proteins it is found in. CBS domains are also found in a wide variety of other proteins such as 611: 595: 587: 489: 848: 1273: 591: 493: 165: 699: 668: 601: 497: 110: 1278: 122: 78: 1311:"Structure of the Bateman2 domain of yeast Snf4: dimeric association and relevance for AMP binding" 1124:"Structure of the Bateman2 domain of yeast Snf4: dimeric association and relevance for AMP binding" 770:"Structure of the Bateman2 domain of yeast Snf4: dimeric association and relevance for AMP binding" 509: 468:
found in a range of proteins in all species from bacteria to humans. It was first identified as a
1851: 1477: 1421: 1375: 1069: 517: 469: 2059: 2018: 1977: 1936: 1900: 1843: 1804: 1768: 1719: 1692: 1636: 1595: 1559: 1518: 1469: 1413: 1367: 1332: 1291: 1246: 1197: 1145: 1104: 1061: 1026: 1004: 985: 936: 914: 895: 868: 826: 791: 758: 711: 623:
CBS domains are often found in proteins that contain other domains. These domains are usually
188: 156: 2049: 2008: 1967: 1928: 1890: 1882: 1835: 1796: 1758: 1750: 1682: 1672: 1628: 1587: 1549: 1508: 1459: 1448:"Crystal structures of the adenylate sensor from fission yeast AMP-activated protein kinase" 1405: 1359: 1322: 1283: 1236: 1228: 1187: 1179: 1135: 1096: 1053: 1016: 975: 967: 926: 860: 818: 781: 735: 679: 481: 148: 630: 513: 655:). Mutations in the gamma subunit of the AMPK enzyme have been shown to lead to familial 1895: 1870: 1687: 1660: 980: 955: 648: 465: 1763: 1738: 1241: 1216: 1192: 1167: 822: 2076: 1309:
Rudolph MJ, Amodeo GA, Iram SH, Hong SP, Pirino G, Carlson M, Tong L (January 2007).
768:
Rudolph MJ, Amodeo GA, Iram SH, Hong SP, Pirino G, Carlson M, Tong L (January 2007).
723: 102: 1661:"Mg2+-sensing mechanism of Mg2+ transporter MgtE probed by molecular dynamics study" 1481: 1379: 1073: 570: 54: 1855: 1425: 144: 971: 66: 586:
It has been shown that CBS domains bind to adenosyl groups in molecules such as
525: 954:
Yusef YR, Zúñiga L, Catalán M, Niemeyer MI, Cid LP, Sepúlveda FV (April 2006).
864: 1932: 1886: 1800: 1659:
Ishitani R, Sugita Y, Dohmae N, Furuya N, Hattori M, Nureki O (October 2008).
1632: 1591: 1554: 1537: 1513: 1496: 1327: 1310: 1140: 1123: 1100: 1021: 931: 786: 769: 667:). Mutations in the CBS domains of the IMPDH enzyme lead to the eye condition 521: 2054: 2037: 1677: 1464: 1447: 2063: 2022: 1981: 1972: 1955: 1940: 1904: 1847: 1808: 1772: 1723: 1696: 1640: 1599: 1563: 1522: 1473: 1417: 1371: 1336: 1295: 1250: 1201: 1149: 1108: 1065: 1030: 989: 940: 915:"Crystal structure of the cytoplasmic domain of the chloride channel ClC-0" 872: 795: 899: 830: 106: 25: 687: 533:. These CBS domain pairs can associate in a head-to-head (i.e. PDB codes 61: 1997:"Myotonia caused by mutations in the muscle chloride channel gene CLCN1" 1839: 1409: 2013: 1996: 85: 1287: 762: 559: 553: 547: 541: 535: 446: 440: 434: 428: 422: 416: 410: 404: 398: 392: 386: 380: 374: 368: 362: 356: 350: 344: 338: 332: 326: 320: 314: 308: 302: 296: 290: 284: 278: 272: 266: 260: 254: 248: 242: 236: 230: 224: 218: 212: 206: 200: 194: 1754: 1363: 1232: 1183: 1057: 731: 624: 176: 90: 1217:"Bateman domains and adenosine derivatives form a binding contract" 1168:"Bateman domains and adenosine derivatives form a binding contract" 849:"CBS domains: structure, function, and pathology in human proteins" 719: 707: 695: 683: 629: 569: 1869:
Lucas M, Kortazar D, Astigarraga E, et al. (October 2008).
739: 727: 715: 703: 691: 672: 664: 652: 138: 49: 2036:
Cleiren E, BĂ©nichou O, Van Hul E, et al. (December 2001).
647:
protein lead to an inherited disorder of the metabolism called
1497:"Structural insight into AMPK regulation: ADP comes into play" 1954:
Shan X, Dunbrack RL, Christopher SA, Kruger WD (March 2001).
1122:
Rudolph MJ, Amodeo GA, Iram SH, et al. (January 2007).
1737:
Scott JW, Hawley SA, Green KA, et al. (January 2004).
508:
The CBS domain is composed of a beta-alpha-beta-beta-alpha
634:
Example protein domains found associated with CBS domains
187: 175: 155: 137: 132: 116: 96: 84: 72: 60: 48: 40: 35: 18: 1538:"AMPK structure and regulation from three angles" 1654: 1652: 1650: 1441: 1439: 1437: 1435: 1161: 1159: 567:, though its functional role is still unknown. 1536:Kemp BE, Oakhill JS, Scott JW (October 2007). 1391: 1389: 842: 840: 1613: 1611: 1609: 8: 1820: 1818: 1495:Jin X, Townley R, Shapiro L (October 2007). 520:that contains a three-stranded antiparallel 605:, the unknown function protein MJ0100 from 551:​) or a head-to-tail (i.e. PDB codes 129: 2053: 2012: 1971: 1894: 1762: 1686: 1676: 1553: 1512: 1463: 1326: 1277: 1240: 1191: 1139: 1020: 979: 930: 785: 847:Ignoul S, Eggermont J (December 2005). 750: 1710:Hattori M, Nureki O (March 2008). "". 678:Humans have a number of voltage-gated 15: 7: 913:Meyer S, Dutzler R (February 2006). 1446:Townley R, Shapiro L (March 2007). 1003:Markovic S, Dutzler R (June 2007). 478:inosine monophosphate dehydrogenase 14: 1875:Acta Crystallographica Section F 24: 700:idiopathic generalised epilepsy 482:voltage gated chloride channels 472:region in 1997 and named after 661:Wolff–Parkinson–White syndrome 1: 853:Am. J. Physiol., Cell Physiol 823:10.1016/S0968-0004(96)30046-7 609:and the regulatory region of 133:Available protein structures: 1665:Proc. Natl. Acad. Sci. U.S.A 972:10.1113/jphysiol.2005.102392 639:Mutations leading to disease 486:AMP-activated protein kinase 657:hypertrophic cardiomyopathy 645:cystathionine beta synthase 575:Multiple sequence alignment 474:cystathionine beta synthase 2099: 1712:Tanpakushitsu Kakusan Koso 865:10.1152/ajpcell.00282.2005 460:In molecular biology, the 1933:10.1016/j.jmb.2010.03.019 1887:10.1107/S1744309108027930 1801:10.1016/j.jmb.2010.03.019 1633:10.1016/j.jmb.2009.12.012 1592:10.1016/j.jmb.2010.03.045 1555:10.1016/j.str.2007.09.006 1514:10.1016/j.str.2007.07.017 1328:10.1016/j.str.2006.11.014 1141:10.1016/j.str.2006.11.014 1101:10.1107/S0907444907009110 1022:10.1016/j.str.2007.04.013 932:10.1016/j.str.2005.10.008 787:10.1016/j.str.2006.11.014 128: 23: 1215:Kemp BE (January 2004). 1166:Kemp BE (January 2004). 1678:10.1073/pnas.0802991105 1465:10.1126/science.1137503 612:Clostridium perfringens 596:electrostatic potential 2055:10.1093/hmg/10.25.2861 1995:Pusch M (April 2002). 1352:Nat. Struct. Mol. Biol 1046:Nat. Struct. Mol. Biol 635: 578: 633: 573: 1973:10.1093/hmg/10.6.635 730:), and mutations in 669:retinitis pigmentosa 602:Thermus thermophilus 498:s-adenosylmethionine 1840:10.1038/nature06161 1410:10.1038/nature06127 1089:Acta Crystallogr. D 811:Trends Biochem. Sci 510:secondary structure 2014:10.1002/humu.10063 636: 619:Associated domains 579: 518:tertiary structure 470:conserved sequence 1881:(Pt 10): 936–41. 1834:(7161): 496–500. 1787:tetraphosphate". 1288:10.1021/bi982858v 615:pyrophosphatase. 458: 457: 454: 453: 182:structure summary 2090: 2068: 2067: 2057: 2033: 2027: 2026: 2016: 1992: 1986: 1985: 1975: 1951: 1945: 1944: 1915: 1909: 1908: 1898: 1866: 1860: 1859: 1822: 1813: 1812: 1783: 1777: 1776: 1766: 1755:10.1172/JCI19874 1734: 1728: 1727: 1707: 1701: 1700: 1690: 1680: 1656: 1645: 1644: 1615: 1604: 1603: 1574: 1568: 1567: 1557: 1533: 1527: 1526: 1516: 1492: 1486: 1485: 1467: 1458:(5819): 1726–9. 1443: 1430: 1429: 1393: 1384: 1383: 1364:10.1038/nsmb1188 1347: 1341: 1340: 1330: 1306: 1300: 1299: 1281: 1272:(15): 4691–700. 1261: 1255: 1254: 1244: 1233:10.1172/JCI20846 1212: 1206: 1205: 1195: 1184:10.1172/JCI20846 1163: 1154: 1153: 1143: 1119: 1113: 1112: 1095:(Pt 5): 587–96. 1084: 1078: 1077: 1058:10.1038/nsmb1188 1041: 1035: 1034: 1024: 1000: 994: 993: 983: 966:(Pt 1): 173–81. 951: 945: 944: 934: 910: 904: 903: 883: 877: 876: 844: 835: 834: 806: 800: 799: 789: 765: 755: 736:Bartter syndrome 718:), mutations in 706:), mutations in 694:), mutations in 680:chloride channel 562: 556: 550: 544: 538: 516:into a globular 512:pattern that is 449: 443: 437: 431: 425: 419: 413: 407: 401: 395: 389: 383: 377: 371: 365: 359: 353: 347: 341: 335: 329: 323: 317: 311: 305: 299: 293: 287: 281: 275: 269: 263: 257: 251: 245: 239: 233: 227: 221: 215: 209: 203: 197: 130: 28: 16: 2098: 2097: 2093: 2092: 2091: 2089: 2088: 2087: 2083:Protein domains 2073: 2072: 2071: 2042:Hum. Mol. Genet 2035: 2034: 2030: 1994: 1993: 1989: 1960:Hum. Mol. Genet 1953: 1952: 1948: 1917: 1916: 1912: 1868: 1867: 1863: 1824: 1823: 1816: 1785: 1784: 1780: 1743:J. Clin. Invest 1736: 1735: 1731: 1714:(in Japanese). 1709: 1708: 1704: 1671:(40): 15393–8. 1658: 1657: 1648: 1617: 1616: 1607: 1576: 1575: 1571: 1535: 1534: 1530: 1507:(10): 1285–95. 1494: 1493: 1489: 1445: 1444: 1433: 1404:(7161): 492–5. 1395: 1394: 1387: 1349: 1348: 1344: 1308: 1307: 1303: 1279:10.1.1.488.2542 1263: 1262: 1258: 1221:J. Clin. Invest 1214: 1213: 1209: 1172:J. Clin. Invest 1165: 1164: 1157: 1121: 1120: 1116: 1086: 1085: 1081: 1043: 1042: 1038: 1002: 1001: 997: 953: 952: 948: 912: 911: 907: 885: 884: 880: 859:(6): C1369–78. 846: 845: 838: 808: 807: 803: 767: 757: 756: 752: 748: 641: 621: 584: 558: 552: 546: 540: 534: 506: 445: 439: 433: 427: 421: 415: 409: 403: 397: 391: 385: 379: 373: 367: 361: 355: 349: 343: 337: 331: 325: 319: 313: 307: 301: 295: 289: 283: 277: 271: 265: 259: 253: 247: 241: 235: 229: 223: 217: 211: 205: 199: 193: 31: 12: 11: 5: 2096: 2094: 2086: 2085: 2075: 2074: 2070: 2069: 2048:(25): 2861–7. 2028: 1987: 1946: 1927:(3): 400–413. 1910: 1861: 1814: 1778: 1729: 1702: 1646: 1605: 1569: 1548:(10): 1161–3. 1528: 1487: 1431: 1385: 1342: 1301: 1256: 1207: 1155: 1114: 1079: 1036: 995: 946: 925:(2): 299–307. 905: 878: 836: 801: 749: 747: 744: 712:Dent's disease 649:homocystinuria 640: 637: 620: 617: 583: 582:Ligand binding 580: 531:Bateman domain 505: 502: 466:protein domain 456: 455: 452: 451: 191: 185: 184: 179: 173: 172: 159: 153: 152: 142: 135: 134: 126: 125: 120: 114: 113: 100: 94: 93: 88: 82: 81: 76: 70: 69: 64: 58: 57: 52: 46: 45: 42: 38: 37: 33: 32: 29: 21: 20: 13: 10: 9: 6: 4: 3: 2: 2095: 2084: 2081: 2080: 2078: 2065: 2061: 2056: 2051: 2047: 2043: 2039: 2032: 2029: 2024: 2020: 2015: 2010: 2007:(4): 423–34. 2006: 2002: 1998: 1991: 1988: 1983: 1979: 1974: 1969: 1966:(6): 635–43. 1965: 1961: 1957: 1950: 1947: 1942: 1938: 1934: 1930: 1926: 1922: 1914: 1911: 1906: 1902: 1897: 1892: 1888: 1884: 1880: 1876: 1872: 1865: 1862: 1857: 1853: 1849: 1845: 1841: 1837: 1833: 1829: 1821: 1819: 1815: 1810: 1806: 1802: 1798: 1795:(3): 400–13. 1794: 1790: 1782: 1779: 1774: 1770: 1765: 1760: 1756: 1752: 1749:(2): 274–84. 1748: 1744: 1740: 1733: 1730: 1725: 1721: 1717: 1713: 1706: 1703: 1698: 1694: 1689: 1684: 1679: 1674: 1670: 1666: 1662: 1655: 1653: 1651: 1647: 1642: 1638: 1634: 1630: 1627:(3): 800–20. 1626: 1622: 1614: 1612: 1610: 1606: 1601: 1597: 1593: 1589: 1585: 1581: 1573: 1570: 1565: 1561: 1556: 1551: 1547: 1543: 1539: 1532: 1529: 1524: 1520: 1515: 1510: 1506: 1502: 1498: 1491: 1488: 1483: 1479: 1475: 1471: 1466: 1461: 1457: 1453: 1449: 1442: 1440: 1438: 1436: 1432: 1427: 1423: 1419: 1415: 1411: 1407: 1403: 1399: 1392: 1390: 1386: 1381: 1377: 1373: 1369: 1365: 1361: 1357: 1353: 1346: 1343: 1338: 1334: 1329: 1324: 1320: 1316: 1312: 1305: 1302: 1297: 1293: 1289: 1285: 1280: 1275: 1271: 1267: 1260: 1257: 1252: 1248: 1243: 1238: 1234: 1230: 1226: 1222: 1218: 1211: 1208: 1203: 1199: 1194: 1189: 1185: 1181: 1177: 1173: 1169: 1162: 1160: 1156: 1151: 1147: 1142: 1137: 1133: 1129: 1125: 1118: 1115: 1110: 1106: 1102: 1098: 1094: 1090: 1083: 1080: 1075: 1071: 1067: 1063: 1059: 1055: 1051: 1047: 1040: 1037: 1032: 1028: 1023: 1018: 1015:(6): 715–25. 1014: 1010: 1006: 999: 996: 991: 987: 982: 977: 973: 969: 965: 961: 957: 950: 947: 942: 938: 933: 928: 924: 920: 916: 909: 906: 901: 897: 893: 889: 882: 879: 874: 870: 866: 862: 858: 854: 850: 843: 841: 837: 832: 828: 824: 820: 816: 812: 805: 802: 797: 793: 788: 783: 779: 775: 771: 764: 760: 754: 751: 745: 743: 741: 737: 733: 729: 725: 724:osteopetrosis 721: 717: 713: 709: 705: 701: 697: 693: 689: 685: 681: 676: 674: 670: 666: 662: 658: 654: 650: 646: 638: 632: 628: 626: 618: 616: 614: 613: 608: 607:M. jannaschii 604: 603: 597: 593: 589: 581: 576: 572: 568: 566: 565:M. jannaschii 561: 555: 549: 543: 537: 532: 527: 523: 519: 515: 511: 503: 501: 499: 495: 491: 487: 483: 479: 475: 471: 467: 463: 448: 442: 436: 430: 424: 418: 412: 406: 400: 394: 388: 382: 376: 370: 364: 358: 352: 346: 340: 334: 328: 322: 316: 310: 304: 298: 292: 286: 280: 274: 268: 262: 256: 250: 244: 238: 232: 226: 220: 214: 208: 202: 196: 192: 190: 186: 183: 180: 178: 174: 171: 167: 163: 160: 158: 154: 150: 146: 143: 140: 136: 131: 127: 124: 121: 119: 115: 112: 108: 104: 101: 99: 95: 92: 89: 87: 83: 80: 77: 75: 71: 68: 65: 63: 59: 56: 53: 51: 47: 43: 39: 34: 27: 22: 17: 2045: 2041: 2031: 2004: 2000: 1990: 1963: 1959: 1949: 1924: 1920: 1913: 1878: 1874: 1864: 1831: 1827: 1792: 1789:J. Mol. Biol 1788: 1781: 1746: 1742: 1732: 1718:(3): 242–8. 1715: 1711: 1705: 1668: 1664: 1624: 1621:J. Mol. Biol 1620: 1586:(1): 53–70. 1583: 1579: 1572: 1545: 1541: 1531: 1504: 1500: 1490: 1455: 1451: 1401: 1397: 1355: 1351: 1345: 1321:(1): 65–74. 1318: 1314: 1304: 1269: 1266:Biochemistry 1265: 1259: 1227:(2): 182–4. 1224: 1220: 1210: 1178:(2): 182–4. 1175: 1171: 1134:(1): 65–74. 1131: 1127: 1117: 1092: 1088: 1082: 1049: 1045: 1039: 1012: 1008: 998: 963: 959: 949: 922: 918: 908: 894:(3): 160–3. 891: 887: 881: 856: 852: 814: 810: 804: 780:(1): 65–74. 777: 773: 753: 734:can lead to 722:can lead to 710:can lead to 698:can lead to 677: 642: 622: 610: 606: 600: 585: 564: 530: 507: 461: 459: 1358:(1): 60–7. 1052:(1): 60–7. 888:J. Mol. Med 817:(1): 12–3. 36:Identifiers 2001:Hum. Mutat 1921:J Mol Biol 1580:J Mol Biol 960:J. Physiol 746:References 462:CBS domain 145:structures 19:CBS domain 1542:Structure 1501:Structure 1315:Structure 1274:CiteSeerX 1128:Structure 1009:Structure 919:Structure 774:Structure 766:​; 625:enzymatic 557:​, 545:​, 539:​, 526:α-helices 524:with two 504:Structure 444:​, 438:​, 432:​, 426:​, 420:​, 414:​, 408:​, 402:​, 396:​, 390:​, 384:​, 378:​, 372:​, 366:​, 360:​, 354:​, 348:​, 342:​, 336:​, 330:​, 324:​, 318:​, 312:​, 306:​, 300:​, 294:​, 288:​, 282:​, 276:​, 270:​, 264:​, 258:​, 252:​, 246:​, 240:​, 234:​, 228:​, 222:​, 216:​, 210:​, 204:​, 198:​, 67:IPR000644 2077:Category 2064:11741829 2023:11933197 1982:11230183 1941:20303981 1905:18931440 1848:17851531 1809:20303981 1773:14722619 1724:18326297 1697:18832160 1641:20026078 1600:20382158 1564:17937905 1523:17937917 1482:38983201 1474:17289942 1418:17851534 1380:20733119 1372:17195847 1337:17223533 1296:10200156 1251:14722609 1202:14722609 1150:17223533 1109:17452784 1074:20733119 1066:17195847 1031:17562318 990:16469788 941:16472749 873:16275737 796:17223533 688:myotonia 686:lead to 162:RCSB PDB 62:InterPro 1896:2564890 1856:4345919 1688:2563093 1619:pair". 1452:Science 1426:4342092 981:1779660 900:9106071 831:9020585 738:(OMIM: 726:(OMIM: 714:(OMIM: 702:(OMIM: 690:(OMIM: 671:(OMIM: 663:(OMIM: 651:(OMIM: 522:β-sheet 450:​ 123:cd02205 91:PS51371 86:PROSITE 55:PF00571 2062:  2021:  1980:  1939:  1903:  1893:  1854:  1846:  1828:Nature 1807:  1771:  1764:311435 1761:  1722:  1695:  1685:  1639:  1598:  1562:  1521:  1480:  1472:  1424:  1416:  1398:Nature 1378:  1370:  1335:  1294:  1276:  1249:  1242:311445 1239:  1200:  1193:311445 1190:  1148:  1107:  1072:  1064:  1029:  988:  978:  939:  898:  871:  829:  794:  740:241200 732:CLCNKB 728:259700 716:300009 704:600699 692:160800 673:180105 665:600858 653:236200 514:folded 177:PDBsum 151:  141:  111:SUPFAM 41:Symbol 1852:S2CID 1478:S2CID 1422:S2CID 1376:S2CID 1070:S2CID 720:CLCN7 708:CLCN5 696:CLCN2 684:CLCN1 659:with 496:, or 464:is a 107:SCOPe 98:SCOP2 74:SMART 2060:PMID 2019:PMID 1978:PMID 1937:PMID 1901:PMID 1844:PMID 1805:PMID 1769:PMID 1720:PMID 1693:PMID 1637:PMID 1596:PMID 1560:PMID 1519:PMID 1470:PMID 1414:PMID 1368:PMID 1333:PMID 1292:PMID 1247:PMID 1198:PMID 1146:PMID 1105:PMID 1062:PMID 1027:PMID 986:PMID 937:PMID 896:PMID 869:PMID 827:PMID 792:PMID 763:2nye 590:and 560:1PBJ 554:1O50 548:2OOX 542:1PVM 536:3KPC 492:and 484:and 447:3ddj 441:2yzq 435:2yzi 429:2yvx 423:2v9j 417:2v92 411:2v8q 405:2rih 399:2rif 393:2rc3 387:2qh1 381:2oux 375:2oox 369:2o16 363:2nye 357:2nyc 351:2j9l 345:2ef7 339:2d4z 333:2cu0 327:1zfj 321:1yav 315:1y5h 309:1xkf 303:1vrd 297:1vr9 291:1pvn 285:1pvm 279:1pbj 273:1o50 267:1nfb 261:1nf7 255:1mew 249:1mei 243:1meh 237:1me9 231:1me8 225:1me7 219:1lrt 213:1jr1 207:1jcn 201:1b3o 195:1ak5 170:PDBj 166:PDBe 149:ECOD 139:Pfam 103:1zfj 50:Pfam 2050:doi 2009:doi 1968:doi 1929:doi 1925:398 1891:PMC 1883:doi 1836:doi 1832:449 1797:doi 1793:398 1759:PMC 1751:doi 1747:113 1683:PMC 1673:doi 1669:105 1629:doi 1625:396 1588:doi 1584:399 1550:doi 1509:doi 1460:doi 1456:315 1406:doi 1402:449 1360:doi 1323:doi 1284:doi 1237:PMC 1229:doi 1225:113 1188:PMC 1180:doi 1176:113 1136:doi 1097:doi 1054:doi 1017:doi 976:PMC 968:doi 964:572 927:doi 861:doi 857:289 819:doi 782:doi 759:PDB 742:). 675:). 592:ATP 588:AMP 494:ATP 490:AMP 189:PDB 157:PDB 118:CDD 79:CBS 44:CBS 2079:: 2058:. 2046:10 2044:. 2040:. 2017:. 2005:19 2003:. 1999:. 1976:. 1964:10 1962:. 1958:. 1935:. 1923:. 1899:. 1889:. 1879:64 1877:. 1873:. 1850:. 1842:. 1830:. 1817:^ 1803:. 1791:. 1767:. 1757:. 1745:. 1741:. 1716:53 1691:. 1681:. 1667:. 1663:. 1649:^ 1635:. 1623:. 1608:^ 1594:. 1582:. 1558:. 1546:15 1544:. 1540:. 1517:. 1505:15 1503:. 1499:. 1476:. 1468:. 1454:. 1450:. 1434:^ 1420:. 1412:. 1400:. 1388:^ 1374:. 1366:. 1356:14 1354:. 1331:. 1319:15 1317:. 1313:. 1290:. 1282:. 1270:38 1268:. 1245:. 1235:. 1223:. 1219:. 1196:. 1186:. 1174:. 1170:. 1158:^ 1144:. 1132:15 1130:. 1126:. 1103:. 1093:63 1091:. 1068:. 1060:. 1050:14 1048:. 1025:. 1013:15 1011:. 1007:. 984:. 974:. 962:. 958:. 935:. 923:14 921:. 917:. 892:75 890:. 867:. 855:. 851:. 839:^ 825:. 815:22 813:. 790:. 778:15 776:. 772:. 761:: 500:. 480:, 168:; 164:; 147:/ 109:/ 105:/ 2066:. 2052:: 2025:. 2011:: 1984:. 1970:: 1943:. 1931:: 1907:. 1885:: 1858:. 1838:: 1811:. 1799:: 1775:. 1753:: 1726:. 1699:. 1675:: 1643:. 1631:: 1602:. 1590:: 1566:. 1552:: 1525:. 1511:: 1484:. 1462:: 1428:. 1408:: 1382:. 1362:: 1339:. 1325:: 1298:. 1286:: 1253:. 1231:: 1204:. 1182:: 1152:. 1138:: 1111:. 1099:: 1076:. 1056:: 1033:. 1019:: 992:. 970:: 943:. 929:: 902:. 875:. 863:: 833:. 821:: 798:. 784::

Index


Pfam
PF00571
InterPro
IPR000644
SMART
CBS
PROSITE
PS51371
SCOP2
1zfj
SCOPe
SUPFAM
CDD
cd02205
Pfam
structures
ECOD
PDB
RCSB PDB
PDBe
PDBj
PDBsum
structure summary
PDB
1ak5
1b3o
1jcn
1jr1
1lrt

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