403:
635:
37:
767:
Erlandsen H, Fusetti F, Martinez A, Hough E, Flatmark T, Stevens RC (December 1997). "Crystal structure of the catalytic domain of human phenylalanine hydroxylase reveals the structural basis for phenylketonuria".
402:
160:
41:
crystal structure of ternary complex of the catalytic domain of human phenylalanine hydroxylase (Fe(II)) complexed with tetrahydrobiopterin and norleucine
691:
for the catalytic core. Thus, by combining with the same C domain, the proteins acquired the unique regulatory properties of the separate R domains.
572:
565:
558:
551:
96:
537:
530:
523:
180:
710:"Full-length cDNA for rabbit tryptophan hydroxylase: functional domains and evolution of aromatic amino acid hydroxylases"
609:
208:
623:
616:
300:
116:
936:
234:
230:
223:
212:
168:
479:
353:
219:
164:
721:
331:
109:
846:
Lindemann L, Hoener MC (May 2005). "A renaissance in trace amines inspired by a novel GPCR family".
121:
372:
276:
793:
672:
335:
17:
898:
863:
828:
785:
749:
516:
155:
890:
855:
820:
777:
739:
729:
489:
280:
147:
680:
320:
304:
284:
245:
881:
Wang X, Li J, Dong G, Yue J (February 2014). "The endogenous substrates of brain CYP2D".
725:
687:
arose from genes that were recruited from different sources to combine with the common
684:
472:
448:
744:
709:
930:
824:
652:
640:
411:
387:
357:
339:
101:
65:
811:
Broadley KJ (March 2010). "The vascular effects of trace amines and amphetamines".
797:
366:
253:
143:
922:
894:
77:
644:
395:
391:
241:
859:
648:
509:
499:
441:
326:
311:
308:
268:
249:
200:
734:
676:
361:
902:
867:
832:
789:
753:
105:
36:
918:
465:
455:
421:
315:
264:
237:
226:
215:
72:
781:
260:
89:
84:
595:
431:
348:
288:
204:
175:
275:
and catalyzes the ring hydroxylation of aromatic amino acids using
914:
688:
634:
272:
137:
60:
671:
It has been suggested that the AAAH enzymes each contain a
259:
The AAAH enzymes are functionally and structurally related
913:
This article incorporates text from the public domain
708:
Grenett HE, Ledley FD, Reed LL, Woo SL (August 1987).
379:
193:
Biopterin-dependent aromatic amino acid hydroxylases
174:
154:
136:
131:
115:
95:
83:
71:
59:
51:
46:
29:
8:
128:
35:
743:
733:
700:
679:(C) domain and an unrelated N-terminal
683:(R) domain. It is possible that the R
26:
283:. The AAAH enzymes are regulated by
7:
848:Trends in Pharmacological Sciences
307:, the most common inborn error of
25:
883:European Journal of Pharmacology
825:10.1016/j.pharmthera.2009.11.005
633:
401:
364:biosynthesis: the conversion of
342:biosynthesis: the conversion of
813:Pharmacology & Therapeutics
18:Aromatic amino acid hydroxylase
1:
132:Available protein structures:
895:10.1016/j.ejphar.2013.12.025
714:Proc. Natl. Acad. Sci. U.S.A
314:. Phenylalanine hydroxylase
271:. Each AAAH enzyme contains
263:which act as rate-limiting
240:). These enzymes primarily
209:phenylalanine 4-hydroxylase
199:) are a family of aromatic
953:
912:
860:10.1016/j.tips.2005.03.007
386:Biosynthetic pathways for
301:phenylalanine hydroxylase
127:
34:
231:tryptophan 5-hydroxylase
735:10.1073/pnas.84.16.5530
643:and phenethylaminergic
373:5-hydroxy-L-tryptophan
354:tryptophan hydroxylase
220:tyrosine 3-hydroxylase
647:are derived from the
483:-Methylphenethylamine
303:deficiency can cause
332:Tyrosine hydroxylase
291:in their N-termini.
782:10.1038/nsb1297-995
726:1987PNAS...84.5530G
277:tetrahydrobiopterin
360:-limiting step in
336:rate-limiting step
318:the conversion of
295:Role in metabolism
269:metabolic pathways
770:Nat. Struct. Biol
663:
662:
517:3-Methoxytyramine
434:
424:
414:
256:, respectively.
190:
189:
186:
185:
181:structure summary
16:(Redirected from
944:
907:
906:
878:
872:
871:
843:
837:
836:
808:
802:
801:
776:(12): 995–1000.
764:
758:
757:
747:
737:
705:
655:
637:
636:
626:
619:
612:
605:
598:
589:
588:
582:
581:
575:
568:
561:
554:
547:
540:
533:
526:
519:
512:
505:
495:
485:
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468:
461:
451:
444:
437:
432:
427:
422:
417:
412:
405:
380:
375:
369:
351:
345:
329:
323:
244:the amino acids
129:
39:
27:
21:
952:
951:
947:
946:
945:
943:
942:
941:
937:Protein domains
927:
926:
925:
911:
910:
880:
879:
875:
845:
844:
840:
810:
809:
805:
766:
765:
761:
707:
706:
702:
697:
685:protein domains
669:
664:
659:
658:
657:
653:L-phenylalanine
651:
638:
630:
629:
628:
627:
622:
620:
615:
613:
608:
606:
603:
601:
599:
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583:
579:
578:
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571:
569:
564:
562:
557:
555:
550:
548:
545:
543:
541:
536:
534:
529:
527:
522:
520:
515:
513:
508:
506:
498:
496:
493:-Methyltyramine
488:
486:
478:
476:
471:
469:
464:
462:
454:
452:
447:
445:
440:
438:
430:
428:
420:
418:
410:
406:
398:
371:
365:
347:
343:
325:
321:L-phenylalanine
319:
305:phenylketonuria
297:
285:phosphorylation
246:L-phenylalanine
207:which includes
42:
23:
22:
15:
12:
11:
5:
950:
948:
940:
939:
929:
928:
909:
908:
873:
854:(5): 274–281.
838:
819:(3): 363–375.
803:
759:
720:(16): 5530–4.
699:
698:
696:
693:
668:
665:
661:
660:
641:catecholamines
632:
631:
621:
614:
607:
600:
591:
584:
577:
570:
563:
556:
549:
542:
535:
528:
521:
514:
507:
497:
487:
477:
473:Norepinephrine
470:
463:
453:
449:Phenethylamine
446:
439:
429:
419:
415:-Phenylalanine
409:
408:
407:
400:
399:
388:catecholamines
385:
384:
383:
378:
356:catalyzes the
334:catalyzes the
296:
293:
267:for important
188:
187:
184:
183:
178:
172:
171:
158:
152:
151:
141:
134:
133:
125:
124:
119:
113:
112:
99:
93:
92:
87:
81:
80:
75:
69:
68:
63:
57:
56:
53:
49:
48:
44:
43:
40:
32:
31:
24:
14:
13:
10:
9:
6:
4:
3:
2:
949:
938:
935:
934:
932:
924:
920:
916:
904:
900:
896:
892:
888:
884:
877:
874:
869:
865:
861:
857:
853:
849:
842:
839:
834:
830:
826:
822:
818:
814:
807:
804:
799:
795:
791:
787:
783:
779:
775:
771:
763:
760:
755:
751:
746:
741:
736:
731:
727:
723:
719:
715:
711:
704:
701:
694:
692:
690:
686:
682:
678:
674:
666:
654:
650:
646:
642:
625:
618:
611:
597:
574:
567:
560:
553:
539:
532:
525:
518:
511:
504:
502:
494:
492:
484:
482:
474:
467:
460:
458:
450:
443:
436:
426:
416:
404:
397:
393:
389:
382:
381:
377:
374:
368:
363:
359:
355:
352:. Similarly,
350:
341:
340:catecholamine
337:
333:
328:
322:
317:
313:
310:
306:
302:
294:
292:
290:
286:
282:
278:
274:
270:
266:
262:
257:
255:
251:
247:
243:
239:
236:
232:
228:
225:
221:
217:
214:
210:
206:
202:
198:
194:
182:
179:
177:
173:
170:
166:
162:
159:
157:
153:
149:
145:
142:
139:
135:
130:
126:
123:
120:
118:
114:
111:
107:
103:
100:
98:
94:
91:
88:
86:
82:
79:
76:
74:
70:
67:
64:
62:
58:
54:
50:
45:
38:
33:
28:
19:
886:
882:
876:
851:
847:
841:
816:
812:
806:
773:
769:
762:
717:
713:
703:
670:
645:trace amines
500:
490:
480:
456:
392:trace amines
367:L-tryptophan
298:
258:
254:L-tryptophan
203:hydroxylase
196:
192:
191:
889:: 211–218.
675:C-terminal
639:In humans,
503:-Octopamine
442:Epinephrine
396:human brain
299:In humans,
279:(BH4) as a
242:hydroxylate
55:Biopterin_H
47:Identifiers
30:Biopterin_H
695:References
681:regulatory
649:amino acid
510:Synephrine
344:L-tyrosine
327:L-tyrosine
312:metabolism
309:amino acid
250:L-tyrosine
201:amino acid
144:structures
923:IPR019774
677:catalytic
673:conserved
667:Structure
459:-Tyramine
425:-Tyrosine
362:serotonin
316:catalyzes
281:substrate
265:catalysts
238:1.14.16.4
227:1.14.16.2
216:1.14.16.1
90:PDOC00316
78:IPR019774
931:Category
919:InterPro
903:24374199
868:15860375
833:19948186
466:Dopamine
261:proteins
161:RCSB PDB
73:InterPro
798:6293946
790:9406548
754:3475690
722:Bibcode
604:pathway
546:pathway
544:primary
394:in the
289:serines
229:), and
205:enzymes
122:cd00361
85:PROSITE
66:PF00351
901:
866:
831:
796:
788:
752:
745:298896
742:
596:CYP2D6
349:L-DOPA
252:, and
176:PDBsum
150:
140:
110:SUPFAM
52:Symbol
794:S2CID
602:minor
593:brain
435:-DOPA
106:SCOPe
97:SCOP2
917:and
915:Pfam
899:PMID
864:PMID
829:PMID
786:PMID
750:PMID
689:gene
610:COMT
587:AAAH
580:AAAH
573:PNMT
566:PNMT
559:PNMT
552:PNMT
538:AADC
531:AADC
524:AADC
390:and
358:rate
273:iron
197:AAAH
169:PDBj
165:PDBe
148:ECOD
138:Pfam
102:1toh
61:Pfam
891:doi
887:724
856:doi
821:doi
817:125
778:doi
740:PMC
730:doi
624:DBH
617:DBH
370:to
346:to
338:in
324:to
287:at
218:),
156:PDB
117:CDD
933::
921::
897:.
885:.
862:.
852:26
850:.
827:.
815:.
792:.
784:.
772:.
748:.
738:.
728:.
718:84
716:.
712:.
376:.
330:.
248:,
235:EC
224:EC
213:EC
167:;
163:;
146:/
108:/
104:/
905:.
893::
870:.
858::
835:.
823::
800:.
780::
774:4
756:.
732::
724::
656:.
501:p
491:N
481:N
457:p
433:L
423:L
413:L
233:(
222:(
211:(
195:(
20:)
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
