76:
1129:
40:
282:
263:
1190:
339:
111:
17:
156:
1166:
420:
806:
667:
625:
528:
690:
332:
988:
425:
151:
1185:
1029:
325:
352:
1003:
998:
1159:
920:
756:
1140:
508:
1007:
1152:
811:
1056:
1024:
892:
695:
1098:
430:
1088:
1067:
739:
348:
213:
186:
1093:
983:
700:
672:
440:
121:
75:
387:
382:
297:
230:
888:
553:
882:
751:
533:
146:
116:
106:
1037:
472:
898:
848:
603:
360:
171:
8:
1103:
954:
843:
503:
458:
435:
286:
317:
838:
761:
728:
397:
191:
176:
1108:
580:
136:
64:
291:
1063:
945:
926:
743:
718:
166:
1051:
1046:
878:
798:
598:
402:
302:
218:
196:
181:
141:
1136:
958:
912:
723:
662:
392:
126:
1179:
993:
852:
785:
640:
161:
69:
276:
272:
829:
645:
575:
570:
513:
101:
978:
821:
131:
1135:
This article about an endocrine, nutritional, or metabolic disease is a
255:
1072:
1041:
833:
710:
682:
450:
364:
208:
89:
48:
88:
are metabolic disorders which impair the synthesis and degradation of
968:
617:
613:
562:
548:
963:
908:
874:
862:
789:
776:
524:
482:
56:
1128:
866:
590:
520:
495:
486:
412:
39:
654:
373:
267:
52:
347:
112:
Branched-chain keto acid dehydrogenase kinase deficiency
245:
1191:Endocrine, nutritional and metabolic disease stubs
480:
202:
157:Carbamoyl-phosphate synthase I deficiency disease
1177:
421:3-hydroxy-3-methylglutaryl-CoA lyase deficiency
224:
807:6-Pyruvoyltetrahydropterin synthase deficiency
1160:
333:
33:Congenital disorders of amino acid metabolism
691:2-Methylbutyryl-CoA dehydrogenase deficiency
989:Carbamoyl phosphate synthetase I deficiency
426:3-Methylcrotonyl-CoA carboxylase deficiency
1167:
1153:
340:
326:
74:
38:
152:Ornithine carbamoyltransferase deficiency
668:Isobutyryl-CoA dehydrogenase deficiency
18:List of amino acid metabolism disorders
14:
1178:
494:
86:Inborn errors of amino acid metabolism
1004:Ornithine transcarbamylase deficiency
999:N-Acetylglutamate synthase deficiency
321:
1123:
921:Dopamine beta hydroxylase deficiency
757:Methylmalonyl-CoA mutase deficiency
24:
25:
1202:
509:Glutathione synthetase deficiency
241:
1127:
1186:Amino acid metabolism disorders
812:Tetrahydrobiopterin deficiency
203:Amino acid transport disorders
47:The general structure of an α-
13:
1:
1057:Lysinuric protein intolerance
431:3-Methylglutaconic aciduria 1
236:
1139:. You can help Knowledge by
696:Beta-ketothiolase deficiency
225:Amino acid storage disorders
7:
1099:Ethylmalonic encephalopathy
10:
1207:
1122:
1089:2-Hydroxyglutaric aciduria
1068:Oculocerebrorenal syndrome
214:Dicarboxylic aminoaciduria
187:Nonketotic hyperglycinemia
55:group on the left and the
1094:Aminoacylase 1 deficiency
1081:
1023:
984:Argininosuccinic aciduria
953:
943:
907:
893:Hermansky–Pudlak syndrome
861:
820:
797:
784:
774:
737:
709:
701:Maple syrup urine disease
681:
673:Maple syrup urine disease
653:
638:
612:
589:
561:
546:
471:
449:
441:Maple syrup urine disease
411:
372:
359:
249:
122:Maple syrup urine disease
63:
46:
37:
32:
383:Glutaric acidemia type 1
231:Glutaric acidemia type 2
95:
889:Oculocutaneous albinism
1008:translocase deficiency
752:Methylmalonic acidemia
534:Glycine encephalopathy
147:Biotinidase deficiency
117:Methylmalonic acidemia
107:Aspartylglucosaminuria
1038:Solute carrier family
353:amino acid metabolism
899:Waardenburg syndrome
849:Tyrosinemia type III
604:Prolidase deficiency
172:Hyperhomocysteinemia
1104:Fumarase deficiency
844:Tyrosinemia type II
504:D-Glyceric acidemia
459:Hypertryptophanemia
436:Isovaleric acidemia
839:Tyrosinemia type I
762:Propionic acidemia
729:Hypermethioninemia
398:Pipecolic acidemia
192:Propionic acidemia
177:Hypermethioninemia
59:group on the right
1148:
1147:
1117:
1116:
1109:Trimethylaminuria
1019:
1018:
1015:
1014:
939:
938:
935:
934:
770:
769:
634:
633:
581:Urocanic aciduria
542:
541:
467:
466:
312:
311:
137:Trimethylaminuria
83:
82:
27:Medical condition
16:(Redirected from
1198:
1169:
1162:
1155:
1131:
1124:
1064:Fanconi syndrome
951:
950:
927:Brunner syndrome
795:
794:
782:
781:
719:Cystathioninuria
651:
650:
559:
558:
492:
491:
478:
477:
370:
369:
342:
335:
328:
319:
318:
247:
246:
167:Hyperargininemia
79:
78:
42:
30:
29:
21:
1206:
1205:
1201:
1200:
1199:
1197:
1196:
1195:
1176:
1175:
1174:
1173:
1120:
1118:
1113:
1077:
1052:Iminoglycinuria
1047:Hartnup disease
1028:
1011:
961:
931:
903:
879:Ocular albinism
857:
816:
799:Phenylketonuria
766:
733:
705:
677:
644:
630:
608:
599:Hyperprolinemia
585:
554:α-ketoglutarate
552:
538:
529:GAMT deficiency
463:
445:
407:
403:Saccharopinuria
376:/straight chain
355:
346:
315:
313:
308:
307:
258:
244:
239:
227:
219:Hartnup disease
205:
197:Hyperprolinemia
182:Hyperlysinemias
142:Hartnup disease
98:
73:
28:
23:
22:
15:
12:
11:
5:
1204:
1194:
1193:
1188:
1172:
1171:
1164:
1157:
1149:
1146:
1145:
1132:
1115:
1114:
1112:
1111:
1106:
1101:
1096:
1091:
1085:
1083:
1079:
1078:
1076:
1075:
1070:
1060:
1059:
1054:
1049:
1044:
1034:
1032:
1021:
1020:
1017:
1016:
1013:
1012:
1010:
1001:
996:
991:
986:
981:
976:
974:
973:
972:
959:Hyperammonemia
948:
941:
940:
937:
936:
933:
932:
930:
929:
923:
917:
915:
913:Norepinephrine
905:
904:
902:
901:
896:
886:
871:
869:
859:
858:
856:
855:
846:
841:
836:
826:
824:
818:
817:
815:
814:
809:
803:
801:
792:
779:
772:
771:
768:
767:
765:
764:
759:
754:
748:
746:
735:
734:
732:
731:
726:
724:Homocystinuria
721:
715:
713:
707:
706:
704:
703:
698:
693:
687:
685:
679:
678:
676:
675:
670:
665:
663:Hypervalinemia
659:
657:
648:
636:
635:
632:
631:
629:
628:
622:
620:
610:
609:
607:
606:
601:
595:
593:
587:
586:
584:
583:
578:
573:
567:
565:
556:
544:
543:
540:
539:
537:
536:
531:
517:
516:
511:
506:
500:
498:
489:
475:
469:
468:
465:
464:
462:
461:
455:
453:
447:
446:
444:
443:
438:
433:
428:
423:
417:
415:
409:
408:
406:
405:
400:
395:
393:Hyperlysinemia
390:
385:
379:
377:
367:
357:
356:
345:
344:
337:
330:
322:
310:
309:
306:
305:
294:
279:
259:
254:
253:
251:
250:Classification
243:
242:External links
240:
238:
235:
234:
233:
226:
223:
222:
221:
216:
211:
204:
201:
200:
199:
194:
189:
184:
179:
174:
169:
164:
159:
154:
149:
144:
139:
134:
129:
127:Homocystinuria
124:
119:
114:
109:
104:
97:
94:
81:
80:
67:
61:
60:
44:
43:
35:
34:
26:
9:
6:
4:
3:
2:
1203:
1192:
1189:
1187:
1184:
1183:
1181:
1170:
1165:
1163:
1158:
1156:
1151:
1150:
1144:
1142:
1138:
1133:
1130:
1126:
1125:
1121:
1110:
1107:
1105:
1102:
1100:
1097:
1095:
1092:
1090:
1087:
1086:
1084:
1080:
1074:
1071:
1069:
1065:
1062:
1061:
1058:
1055:
1053:
1050:
1048:
1045:
1043:
1039:
1036:
1035:
1033:
1031:
1026:
1022:
1009:
1005:
1002:
1000:
997:
995:
994:Citrullinemia
992:
990:
987:
985:
982:
980:
977:
975:
970:
967:
966:
965:
960:
956:
952:
949:
947:
942:
928:
924:
922:
919:
918:
916:
914:
910:
906:
900:
897:
894:
890:
887:
884:
880:
876:
873:
872:
870:
868:
864:
860:
854:
853:Hawkinsinuria
850:
847:
845:
842:
840:
837:
835:
831:
828:
827:
825:
823:
819:
813:
810:
808:
805:
804:
802:
800:
796:
793:
791:
787:
786:Phenylalanine
783:
780:
778:
773:
763:
760:
758:
755:
753:
750:
749:
747:
745:
741:
736:
730:
727:
725:
722:
720:
717:
716:
714:
712:
708:
702:
699:
697:
694:
692:
689:
688:
686:
684:
680:
674:
671:
669:
666:
664:
661:
660:
658:
656:
652:
649:
647:
642:
641:propionyl-CoA
637:
627:
624:
623:
621:
619:
615:
611:
605:
602:
600:
597:
596:
594:
592:
588:
582:
579:
577:
574:
572:
569:
568:
566:
564:
560:
557:
555:
550:
545:
535:
532:
530:
526:
522:
519:
518:
515:
512:
510:
507:
505:
502:
501:
499:
497:
493:
490:
488:
484:
479:
476:
474:
470:
460:
457:
456:
454:
452:
448:
442:
439:
437:
434:
432:
429:
427:
424:
422:
419:
418:
416:
414:
410:
404:
401:
399:
396:
394:
391:
389:
386:
384:
381:
380:
378:
375:
371:
368:
366:
362:
358:
354:
350:
343:
338:
336:
331:
329:
324:
323:
320:
316:
304:
300:
299:
295:
293:
289:
288:
284:
280:
278:
274:
270:
269:
265:
261:
260:
257:
252:
248:
232:
229:
228:
220:
217:
215:
212:
210:
207:
206:
198:
195:
193:
190:
188:
185:
183:
180:
178:
175:
173:
170:
168:
165:
163:
162:Citrullinemia
160:
158:
155:
153:
150:
148:
145:
143:
140:
138:
135:
133:
130:
128:
125:
123:
120:
118:
115:
113:
110:
108:
105:
103:
100:
99:
93:
91:
87:
77:
71:
70:Endocrinology
68:
66:
62:
58:
54:
50:
45:
41:
36:
31:
19:
1141:expanding it
1134:
1119:
946:oxaloacetate
830:Alkaptonuria
646:succinyl-CoA
576:Histidinemia
571:Carnosinemia
514:Sarcosinemia
349:Inborn error
314:
296:
281:
262:
102:Alkaptonuria
85:
84:
979:Argininemia
822:Tyrosinemia
132:Tyrosinemia
90:amino acids
51:, with the
1180:Categories
1073:Cystinosis
1042:Cystinuria
955:Urea cycle
834:Ochronosis
711:Methionine
683:Isoleucine
451:Tryptophan
365:acetyl-CoA
237:References
209:Cystinuria
49:amino acid
1030:IE of RTT
1025:Transport
969:aspartate
925:reverse:
618:glutamine
614:Glutamate
563:Histidine
549:glutamate
65:Specialty
964:arginine
909:Tyrosine
875:Albinism
863:Tyrosine
790:tyrosine
777:fumarate
738:General
525:Creatine
483:pyruvate
57:carboxyl
867:Melanin
591:Proline
521:Glycine
496:Glycine
487:citrate
413:Leucine
303:D000592
655:Valine
626:SSADHD
388:type 2
374:Lysine
72:
1082:Other
96:Types
53:amino
1137:stub
298:MeSH
287:9-CM
351:of
292:270
283:ICD
277:E72
273:E70
264:ICD
1182::
1066::
1040::
944:G→
877::
775:G→
744:OA
740:BC
639:G→
547:G→
527::
481:G→
301::
290::
271::
268:10
92:.
1168:e
1161:t
1154:v
1143:.
1027:/
1006:/
971:)
962:(
957:/
911:→
895:)
891:(
885:)
883:1
881:(
865:→
851:/
832:/
788:/
742:/
643:→
616:/
551:→
523:→
485:→
473:G
363:→
361:K
341:e
334:t
327:v
285:-
275:-
266:-
256:D
20:)
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
