27:
210:. Although MLCL AT-1 and MTP are different proteins, in 2012 the same lab discovered that MTP did indeed have cardiolipin remodeling capabilities. This suggests a possible link between mitochondrial membrane cardiolipin content and
129:
between the two enzymes. This is especially interesting, because up until then it was unknown exactly how MTP was associated with the inner mitochondrial membrane, and this discovery may provide the explanation.
231:
41:
718:
868:
86:
1262:
610:
226:
1743:
1738:
20:
1829:
1411:
1497:
861:
509:
Beh, Christopher; Taylor, William A.; Mejia, Edgard M.; Mitchell, Ryan W.; Choy, Patrick C.; Sparagna, Genevieve C.; Hatch, Grant M. (2012).
1306:
846:
1255:
1184:
1039:
603:
191:
163:
1597:
1532:
998:
696:
90:
82:
1542:
1367:
1225:
1044:
854:
334:
Chocron, E. S.; Sayre, N. L.; Holstein, D.; Saelim, N.; Ibdah, J. A.; Dong, L. Q.; Zhu, X.; Cheng, S.-Y.; Lechleiter, J. D. (2012).
1868:
1666:
1301:
1034:
2035:
1863:
1527:
1248:
1110:
761:
1858:
1644:
1602:
1026:
766:
738:
711:
689:
674:
639:
596:
236:
1448:
1390:
1280:
1127:
684:
55:
1502:
958:
953:
1676:
1671:
1475:
1341:
948:
578:
1401:
1207:
1168:
1150:
982:
277:"Evidence for Physical Association of Mitochondrial Fatty Acid Oxidation and Oxidative Phosphorylation Complexes"
102:
452:"Identification of the Human Mitochondrial Linoleoyl-coenzyme A Monolysocardiolipin Acyltransferase (MLCL AT-1)"
1592:
1470:
788:
582:
336:"The Trifunctional Protein Mediates Thyroid Hormone Receptor-Dependent Stimulation of Mitochondria Metabolism"
1639:
1426:
1003:
820:
798:
778:
171:
1634:
1291:
1069:
1017:
925:
783:
679:
2008:
1748:
1624:
1460:
1438:
885:
393:"Estrogen Receptor Alpha Interacts with Mitochondrial Protein HADHB and Affects Beta-Oxidation Activity"
126:
1849:
1755:
1612:
1561:
1507:
935:
522:
256:"Long-Chain Acyl CoA Dehydrogenase Deficiency: eMedicine Pediatrics: Genetics and Metabolic Disease"
1145:
1099:
963:
940:
835:
825:
793:
207:
195:
1779:
1547:
1421:
830:
2055:
1769:
1572:
1353:
920:
558:
540:
491:
473:
432:
414:
373:
355:
316:
298:
167:
143:
1582:
1375:
881:
548:
530:
481:
463:
422:
404:
363:
347:
306:
288:
1487:
1380:
623:
151:
526:
255:
1160:
1137:
1054:
771:
654:
631:
553:
510:
486:
451:
427:
392:
368:
335:
311:
276:
211:
63:
37:
2049:
1271:
666:
649:
1904:
1879:
1537:
1416:
1329:
889:
122:
106:
31:
511:"Human Trifunctional Protein Alpha Links Cardiolipin Remodeling to Beta-Oxidation"
588:
535:
911:
644:
203:
114:
26:
1656:
1318:
1199:
974:
893:
877:
756:
733:
275:
Wang, Y.; Mohsen, A.-W.; Mihalik, S. J.; Goetzman, E. S.; Vockley, J. (2010).
199:
155:
42:
long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, LCHAD deficiency
34:
544:
477:
418:
359:
302:
1587:
468:
409:
293:
125:. Not only does MTP appear to be bound to Complex I, but it also appears to
110:
59:
562:
495:
436:
377:
320:
1733:
1607:
1240:
1217:
351:
175:
139:
66:. MTP is a hetero-octamer composed of four alpha and four beta subunits:
1893:
1888:
1808:
51:
1998:
1993:
1963:
1958:
1838:
1818:
1813:
1803:
1798:
1793:
1788:
1093:
1088:
896:
728:
723:
619:
121:
discovered a functional and physical association between MTP and ETC
2018:
2013:
2003:
1988:
1983:
1978:
1973:
1968:
1953:
1948:
1943:
1938:
1933:
1928:
1923:
1918:
1913:
1709:
1696:
1120:
1115:
1083:
1078:
1073:
813:
808:
75:
70:
25:
138:
Recent research has revealed that MTP can be affected by various
706:
701:
1244:
850:
592:
109:. Reducing equivalents from FAO enter OXPHOS at the level of
170:
to identify MTP as one of the proteins that interacts with
719:
Branched-chain alpha-keto acid dehydrogenase complex
1902:
1877:
1847:
1827:
1777:
1768:
1722:
1685:
1653:
1621:
1569:
1560:
1516:
1484:
1457:
1435:
1398:
1389:
1366:
1338:
1315:
1288:
1279:
1216:
1197:
1177:
1159:
1136:
1062:
1053:
1025:
1016:
991:
972:
934:
910:
903:
821:
Phosphoenolpyruvate sugar phosphotransferase system
749:
665:
630:
87:long-chain 3-hydroxy acyl-coenzyme A dehydrogenase
105:(OXPHOS) are two major metabolic pathways in the
640:Photosynthetic reaction center complex proteins
192:monolysocardiolipin acyltransferase (MLCL AT-1)
227:Mitochondrial trifunctional protein deficiency
1256:
862:
604:
202:end of MTP, linking MTP to the remodeling of
97:Association with the electron transport chain
8:
1774:
1744:Mitochondrial permeability transition pore
1726:
1566:
1395:
1285:
1263:
1249:
1241:
1059:
1022:
907:
869:
855:
847:
611:
597:
589:
190:identified a human mitochondrial protein,
581:at the U.S. National Library of Medicine
552:
534:
485:
467:
426:
408:
367:
310:
292:
48:Mitochondrial trifunctional protein (MTP)
1739:Mitochondrial membrane transport protein
21:microsomal triglyceride transfer protein
247:
1498:Cholesterol side-chain cleavage enzyme
579:mitochondrial+trifunctional+protein+TP
146:located in the mitochondria. Chochron
7:
1185:Hydroxyacyl-Coenzyme A dehydrogenase
450:Taylor, W. A.; Hatch, G. M. (2009).
101:Fatty acid beta-oxidation (FAO) and
16:Inner mitochondrial membrane protein
1412:Coenzyme Q – cytochrome c reductase
1040:Carnitine-acylcarnitine translocase
804:Mitochondrial trifunctional protein
397:Molecular & Cellular Proteomics
391:Zhou, Z.; Zhou, J.; Du, Y. (2012).
222:Disorders associated with MTP are:
158:in a pathway mediated by MTP. Zhou
1598:Oxoglutarate dehydrogenase complex
1533:Glycerol-3-phosphate dehydrogenase
999:Glycerol-3-phosphate dehydrogenase
91:long-chain 3-ketoacyl CoA thiolase
83:2-enoyl coenzyme A (CoA) hydratase
14:
1543:Carnitine palmitoyltransferase II
1226:Long-chain-aldehyde dehydrogenase
1045:Carnitine palmitoyltransferase II
1667:Carbamoyl phosphate synthetase I
1307:Long-chain-fatty-acid—CoA ligase
1302:Carnitine palmitoyltransferase I
1035:Carnitine palmitoyltransferase I
1528:Glutamate aspartate transporter
762:Carbamoyl phosphate synthase II
456:Journal of Biological Chemistry
281:Journal of Biological Chemistry
62:three out of the four steps in
1645:Pyruvate dehydrogenase complex
1603:Succinyl coenzyme A synthetase
767:Aspartate carbamoyltransferase
675:Pyruvate dehydrogenase complex
237:Acute fatty liver of pregnancy
1:
799:Glycine decarboxylase complex
794:Fatty acid synthetase complex
1449:Dihydroorotate dehydrogenase
1128:Acetyl-CoA C-acyltransferase
536:10.1371/journal.pone.0048628
56:inner mitochondrial membrane
1503:Steroid 11-beta-hydroxylase
959:3-Hydroxyacyl ACP dehydrase
2072:
1677:N-Acetylglutamate synthase
1672:Ornithine transcarbamylase
1476:Glycerol phosphate shuttle
1342:monoamine neurotransmitter
949:Beta-ketoacyl-ACP synthase
831:Sucrase-isomaltase complex
697:Oxoglutarate dehydrogenase
174:, a receptor triggered by
18:
2031:
1729:
1705:
1402:oxidative phosphorylation
1208:Malonyl-CoA decarboxylase
1169:Propionyl-CoA carboxylase
1151:2,4 Dienoyl-CoA reductase
983:Stearoyl-CoA desaturase-1
154:stimulates mitochondrial
150:(2012) demonstrated that
103:oxidative phosphorylation
1593:Isocitrate dehydrogenase
1471:Malate-aspartate shuttle
954:Β-Ketoacyl ACP reductase
789:Electron transport chain
583:Medical Subject Headings
81:The three functions are
30:Schematic demonstrating
19:Not to be confused with
1640:Glutamate dehydrogenase
1427:Succinate dehydrogenase
779:P450-containing systems
469:10.1074/jbc.M109.048322
410:10.1074/mcp.M111.011056
340:Molecular Endocrinology
294:10.1074/jbc.M110.139493
194:, that is identical in
2036:mitochondrial diseases
1635:Aspartate transaminase
1292:fatty acid degradation
1070:Acyl CoA dehydrogenase
926:Acetyl-CoA carboxylase
784:Cytochrome b6f complex
182:Cardiolipin remodeling
164:2D gel electrophoresis
44:
1749:Mitochondrial carrier
1625:anaplerotic reactions
1461:mitochondrial shuttle
1439:pyrimidine metabolism
886:fatty acid metabolism
624:multienzyme complexes
218:Clinical significance
29:
1756:Translocator protein
1613:Malate dehydrogenase
1508:Aldosterone synthase
936:Fatty acid synthesis
352:10.1210/me.2011-1348
1368:Intermembrane space
1146:Enoyl CoA isomerase
1100:Enoyl-CoA hydratase
964:Enoyl ACP reductase
941:Fatty acid synthase
836:Tryptophan synthase
826:Polyketide synthase
527:2012PLoSO...748628T
462:(44): 30360–30371.
287:(39): 29834–29841.
208:monolysocardiolipin
196:amino acid sequence
134:Hormonal influences
1723:Other/to be sorted
1688:alcohol metabolism
1548:Uncoupling protein
1422:NADH dehydrogenase
403:(7): M111.011056.
127:channel substrates
45:
2043:
2042:
2027:
2026:
1770:Mitochondrial DNA
1764:
1763:
1718:
1717:
1573:citric acid cycle
1556:
1555:
1362:
1361:
1354:Monoamine oxidase
1238:
1237:
1234:
1233:
1193:
1192:
1012:
1011:
921:ATP citrate lyase
844:
843:
168:mass spectrometry
144:hormone receptors
2063:
1907:
1882:
1852:
1832:
1782:
1775:
1727:
1690:
1660:
1628:
1583:Citrate synthase
1576:
1567:
1521:
1491:
1464:
1442:
1405:
1396:
1376:Adenylate kinase
1347:
1323:
1295:
1286:
1265:
1258:
1251:
1242:
1060:
1023:
992:Triacyl glycerol
908:
882:lipid metabolism
871:
864:
857:
848:
613:
606:
599:
590:
567:
566:
556:
538:
506:
500:
499:
489:
471:
447:
441:
440:
430:
412:
388:
382:
381:
371:
346:(7): 1117–1128.
331:
325:
324:
314:
296:
272:
266:
265:
263:
262:
252:
232:LCHAD deficiency
186:In 2009, Taylor
117:. In 2010, Wang
54:attached to the
2071:
2070:
2066:
2065:
2064:
2062:
2061:
2060:
2046:
2045:
2044:
2039:
2023:
1903:
1898:
1878:
1873:
1848:
1843:
1828:
1823:
1778:
1760:
1714:
1701:
1686:
1681:
1654:
1649:
1622:
1617:
1570:
1552:
1517:
1512:
1488:steroidogenesis
1485:
1480:
1458:
1453:
1436:
1431:
1399:
1385:
1381:Creatine kinase
1358:
1344:
1339:
1334:
1316:
1311:
1289:
1275:
1269:
1239:
1230:
1212:
1189:
1173:
1155:
1132:
1049:
1008:
987:
968:
939:
930:
899:
875:
845:
840:
745:
661:
626:
617:
575:
570:
508:
507:
503:
449:
448:
444:
390:
389:
385:
333:
332:
328:
274:
273:
269:
260:
258:
254:
253:
249:
245:
220:
184:
152:thyroid hormone
136:
99:
40:and effects of
24:
17:
12:
11:
5:
2069:
2067:
2059:
2058:
2048:
2047:
2041:
2040:
2032:
2029:
2028:
2025:
2024:
2022:
2021:
2016:
2011:
2006:
2001:
1996:
1991:
1986:
1981:
1976:
1971:
1966:
1961:
1956:
1951:
1946:
1941:
1936:
1931:
1926:
1921:
1916:
1910:
1908:
1900:
1899:
1897:
1896:
1891:
1885:
1883:
1875:
1874:
1872:
1871:
1866:
1861:
1855:
1853:
1845:
1844:
1842:
1841:
1835:
1833:
1825:
1824:
1822:
1821:
1816:
1811:
1806:
1801:
1796:
1791:
1785:
1783:
1772:
1766:
1765:
1762:
1761:
1759:
1758:
1753:
1752:
1751:
1746:
1736:
1730:
1724:
1720:
1719:
1716:
1715:
1713:
1712:
1706:
1703:
1702:
1700:
1699:
1693:
1691:
1683:
1682:
1680:
1679:
1674:
1669:
1663:
1661:
1651:
1650:
1648:
1647:
1642:
1637:
1631:
1629:
1619:
1618:
1616:
1615:
1610:
1605:
1600:
1595:
1590:
1585:
1579:
1577:
1564:
1558:
1557:
1554:
1553:
1551:
1550:
1545:
1540:
1535:
1530:
1524:
1522:
1514:
1513:
1511:
1510:
1505:
1500:
1494:
1492:
1482:
1481:
1479:
1478:
1473:
1467:
1465:
1455:
1454:
1452:
1451:
1445:
1443:
1433:
1432:
1430:
1429:
1424:
1419:
1414:
1408:
1406:
1393:
1391:Inner membrane
1387:
1386:
1384:
1383:
1378:
1372:
1370:
1364:
1363:
1360:
1359:
1357:
1356:
1350:
1348:
1336:
1335:
1333:
1332:
1326:
1324:
1313:
1312:
1310:
1309:
1304:
1298:
1296:
1283:
1281:Outer membrane
1277:
1276:
1270:
1268:
1267:
1260:
1253:
1245:
1236:
1235:
1232:
1231:
1229:
1228:
1222:
1220:
1214:
1213:
1211:
1210:
1204:
1202:
1195:
1194:
1191:
1190:
1188:
1187:
1181:
1179:
1175:
1174:
1172:
1171:
1165:
1163:
1157:
1156:
1154:
1153:
1148:
1142:
1140:
1134:
1133:
1131:
1130:
1124:
1123:
1118:
1113:
1103:
1102:
1097:
1091:
1086:
1081:
1076:
1066:
1064:
1057:
1055:Beta oxidation
1051:
1050:
1048:
1047:
1042:
1037:
1031:
1029:
1027:Acyl transport
1020:
1014:
1013:
1010:
1009:
1007:
1006:
1001:
995:
993:
989:
988:
986:
985:
979:
977:
970:
969:
967:
966:
961:
956:
951:
945:
943:
932:
931:
929:
928:
923:
917:
915:
905:
901:
900:
876:
874:
873:
866:
859:
851:
842:
841:
839:
838:
833:
828:
823:
818:
817:
816:
811:
801:
796:
791:
786:
781:
776:
775:
774:
772:Dihydroorotase
769:
764:
753:
751:
747:
746:
744:
743:
742:
741:
736:
731:
726:
716:
715:
714:
709:
704:
694:
693:
692:
687:
682:
671:
669:
663:
662:
660:
659:
658:
657:
652:
642:
636:
634:
632:Photosynthesis
628:
627:
618:
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601:
593:
587:
586:
574:
573:External links
571:
569:
568:
521:(11): e48628.
501:
442:
383:
326:
267:
246:
244:
241:
240:
239:
234:
229:
219:
216:
212:beta oxidation
198:to the 59-kDa
183:
180:
135:
132:
98:
95:
79:
78:
73:
64:beta oxidation
38:beta-oxidation
15:
13:
10:
9:
6:
4:
3:
2:
2068:
2057:
2054:
2053:
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1711:
1708:
1707:
1704:
1698:
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1684:
1678:
1675:
1673:
1670:
1668:
1665:
1664:
1662:
1659:
1658:
1652:
1646:
1643:
1641:
1638:
1636:
1633:
1632:
1630:
1627:
1626:
1620:
1614:
1611:
1609:
1606:
1604:
1601:
1599:
1596:
1594:
1591:
1589:
1586:
1584:
1581:
1580:
1578:
1575:
1574:
1568:
1565:
1563:
1559:
1549:
1546:
1544:
1541:
1539:
1536:
1534:
1531:
1529:
1526:
1525:
1523:
1520:
1515:
1509:
1506:
1504:
1501:
1499:
1496:
1495:
1493:
1490:
1489:
1483:
1477:
1474:
1472:
1469:
1468:
1466:
1463:
1462:
1456:
1450:
1447:
1446:
1444:
1441:
1440:
1434:
1428:
1425:
1423:
1420:
1418:
1415:
1413:
1410:
1409:
1407:
1404:
1403:
1397:
1394:
1392:
1388:
1382:
1379:
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1374:
1373:
1371:
1369:
1365:
1355:
1352:
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1349:
1346:
1343:
1337:
1331:
1328:
1327:
1325:
1322:
1320:
1314:
1308:
1305:
1303:
1300:
1299:
1297:
1294:
1293:
1287:
1284:
1282:
1278:
1273:
1272:Mitochondrial
1266:
1261:
1259:
1254:
1252:
1247:
1246:
1243:
1227:
1224:
1223:
1221:
1219:
1215:
1209:
1206:
1205:
1203:
1201:
1196:
1186:
1183:
1182:
1180:
1176:
1170:
1167:
1166:
1164:
1162:
1158:
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667:Dehydrogenase
664:
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629:
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36:
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32:mitochondrial
28:
22:
2033:
1880:ATP synthase
1687:
1655:
1623:
1571:
1538:ATP synthase
1518:
1486:
1459:
1437:
1417:Cytochrome c
1400:
1340:
1330:Kynureninase
1317:
1290:
1106:
890:triglyceride
803:
518:
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459:
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343:
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259:. Retrieved
250:
221:
187:
185:
162:(2012) used
159:
147:
137:
123:respirasomes
118:
107:mitochondria
100:
80:
47:
46:
1830:Complex III
1138:Unsaturated
1018:Degradation
975:desaturases
973:Fatty acid
912:Malonyl-CoA
645:Photosystem
204:cardiolipin
111:complexes I
1850:Complex IV
1657:urea cycle
1345:metabolism
1321:metabolism
1319:tryptophan
1200:acetyl-CoA
1004:Thiokinase
894:fatty acid
878:Metabolism
261:2009-07-11
243:References
200:C-terminal
156:metabolism
35:fatty acid
2034:see also
1780:Complex I
1588:Aconitase
1218:Aldehydes
1161:Odd chain
914:synthesis
904:Synthesis
545:1932-6203
478:0021-9258
419:1535-9476
360:0888-8809
303:0021-9258
60:catalyzes
2056:Proteins
2050:Category
1734:Frataxin
1608:Fumarase
1274:proteins
563:23152787
515:PLOS ONE
496:19737925
437:22375075
378:22570332
321:20663895
176:estrogen
172:ER alpha
140:hormones
1894:MT-ATP8
1889:MT-ATP6
1809:MT-ND4L
1063:General
897:enzymes
620:Enzymes
554:3494688
523:Bibcode
487:2781591
428:3394935
369:3385793
312:2943265
52:protein
1999:MT-TS2
1994:MT-TS1
1964:MT-TL2
1959:MT-TL1
1869:MT-CO3
1864:MT-CO2
1859:MT-CO1
1839:MT-CYB
1819:MT-ND6
1814:MT-ND5
1804:MT-ND4
1799:MT-ND3
1794:MT-ND2
1789:MT-ND1
1562:Matrix
1094:ACADSB
1089:ACADVL
729:BCKDHB
724:BCKDHA
585:(MeSH)
561:
551:
543:
494:
484:
476:
435:
425:
417:
376:
366:
358:
319:
309:
301:
188:et al.
160:et al.
148:et al.
142:, via
119:et al.
58:which
2019:MT-TY
2014:MT-TW
2009:MT-TV
2004:MT-TT
1989:MT-TR
1984:MT-TQ
1979:MT-TP
1974:MT-TN
1969:MT-TM
1954:MT-TK
1949:MT-TI
1944:MT-TH
1939:MT-TG
1934:MT-TF
1929:MT-TE
1924:MT-TD
1919:MT-TC
1914:MT-TA
1710:PMPCB
1697:ALDH2
1519:other
1178:Other
1121:HADHB
1116:HADHA
1084:ACADS
1079:ACADM
1074:ACADL
814:HADHB
809:HADHA
750:Other
206:from
76:HADHB
71:HADHA
50:is a
1905:tRNA
1111:HADH
892:and
707:DLST
702:OGDH
559:PMID
541:ISSN
492:PMID
474:ISSN
433:PMID
415:ISSN
374:PMID
356:ISSN
317:PMID
299:ISSN
166:and
113:and
89:and
1198:To
1107:MTP
757:CAD
739:DLD
734:DBT
712:DLD
549:PMC
531:doi
482:PMC
464:doi
460:284
423:PMC
405:doi
364:PMC
348:doi
307:PMC
289:doi
285:285
115:III
2052::
1109::
888:,
884:/
880::
690:E3
685:E2
680:E1
655:II
622::
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344:26
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93:.
85:,
1264:e
1257:t
1250:v
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1072:(
938:/
870:e
863:t
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650:I
612:e
605:t
598:v
565:.
533::
525::
519:7
498:.
466::
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407::
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350::
323:.
291::
264:.
23:.
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