ID 4.2.1.96 DE 4a-hydroxytetrahydrobiopterin dehydratase. AN 4alpha-hydroxy-tetrahydropterin dehydratase. AN pterin-4alpha-carbinolamine dehydratase. AN tetrahydrobiopterin dehydratase. CA (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin = (6R)-L- CA erythro-6,7-dihydrobiopterin + H2O. CC -!- In concert with EC 1.5.1.34 the enzyme recycles CC 4a-hydroxytetrahydrobiopterin back to tetrahydrobiopterin, CC a cosubstrate for several enzymes, including aromatic amino acid CC hydroxylases. CC -!- The enzyme is bifunctional, and also acts as a dimerization cofactor CC of hepatocyte nuclear factor-1alpha (HNF-1). DR Q9LU63, ATP1_ARATH ; Q6QJ72, PDL2_ARATH ; Q475Y4, PHS1_CUPPJ ; DR Q7NM43, PHS1_GLOVI ; Q9DG45, PHS2_CHICK ; Q46VT8, PHS2_CUPPJ ; DR Q7NK35, PHS2_GLOVI ; Q9H0N5, PHS2_HUMAN ; Q9CZL5, PHS2_MOUSE ; DR Q5R7K1, PHS2_PONAB ; C1F425, PHS_ACIC5 ; A5FWK8, PHS_ACICJ ; DR A0KJG4, PHS_AERHH ; A4SNJ1, PHS_AERS4 ; B6JAT9, PHS_AFIC5 ; DR Q8UC22, PHS_AGRFC ; A6TVX6, PHS_ALKMQ ; B9JUA0, PHS_ALLAM ; DR B4RXT2, PHS_ALTMD ; O66462, PHS_AQUAE ; Q5P905, PHS_AROAE ; DR A1K7V8, PHS_AZOSB ; C1DQH9, PHS_AZOVD ; P61732, PHS_BDEBA ; DR Q2KXB6, PHS_BORA1 ; Q7WG14, PHS_BORBR ; Q7W4J0, PHS_BORPA ; DR Q7VU17, PHS_BORPE ; Q3ZBD3, PHS_BOVIN ; Q89WZ6, PHS_BRADU ; DR A5E8P9, PHS_BRASB ; A4YJY8, PHS_BRASO ; B2S7X9, PHS_BRUA1 ; DR Q2YNV0, PHS_BRUA2 ; A6WV21, PHS_BRUA4 ; Q57FT6, PHS_BRUAB ; DR A9M6T4, PHS_BRUC2 ; C0RGE2, PHS_BRUMB ; P65722, PHS_BRUME ; DR A5VN26, PHS_BRUO2 ; B0CIJ4, PHS_BRUSI ; P65723, PHS_BRUSU ; DR B1YQ64, PHS_BURA4 ; A0K2V4, PHS_BURCH ; B4E566, PHS_BURCJ ; DR Q0BJP4, PHS_BURCM ; Q39L06, PHS_BURL3 ; A9AJD5, PHS_BURM1 ; DR Q62FU4, PHS_BURMA ; B1K1I7, PHS_BURO0 ; Q3JXT0, PHS_BURP1 ; DR Q63PF1, PHS_BURPS ; A4JA07, PHS_BURVG ; Q9TZH6, PHS_CAEEL ; DR B0T207, PHS_CAUSK ; Q9ABI2, PHS_CAUVC ; B8GYB5, PHS_CAUVN ; DR Q3IZF4, PHS_CERS4 ; Q11D07, PHS_CHESB ; O73930, PHS_CHICK ; DR A9WI69, PHS_CHLAA ; B8G3F7, PHS_CHLAD ; B3ECB9, PHS_CHLL2 ; DR Q3B6N5, PHS_CHLL3 ; B3QQ83, PHS_CHLP8 ; B9LCC8, PHS_CHLSY ; DR Q8KFI4, PHS_CHLTE ; A0M1H4, PHS_CHRFK ; Q1QXY2, PHS_CHRSD ; DR Q7NVH7, PHS_CHRVO ; Q47XN8, PHS_COLP3 ; B8HU56, PHS_CYAP4 ; DR Q11PN9, PHS_CYTH3 ; Q47IX1, PHS_DECAR ; Q54RY8, PHS_DICDI ; DR A8LKP0, PHS_DINSH ; O76454, PHS_DROME ; P58249, PHS_DROVI ; DR Q2SCE8, PHS_HAHCH ; A1WWH9, PHS_HALHL ; B9LPE3, PHS_HALLT ; DR Q5V1I9, PHS_HALMA ; B0R6B9, PHS_HALS3 ; Q9HP73, PHS_HALSA ; DR A9AXS2, PHS_HERA2 ; P61457, PHS_HUMAN ; Q31HZ6, PHS_HYDCU ; DR P0C8L6, PHS_HYPEX ; Q5QU19, PHS_IDILO ; Q28VA7, PHS_JANSC ; DR Q1IP68, PHS_KORVE ; Q5X3Q4, PHS_LEGPA ; A5IDI1, PHS_LEGPC ; DR Q5ZU09, PHS_LEGPH ; Q5WV42, PHS_LEGPL ; A6VSD2, PHS_MARMS ; DR A1TZA3, PHS_MARN8 ; Q60BH8, PHS_METCA ; A4YEM3, PHS_METS5 ; DR B0JQV0, PHS_MICAN ; P61458, PHS_MOUSE ; A0QCA6, PHS_MYCA1 ; DR B1MLG8, PHS_MYCA9 ; P0A5S3, PHS_MYCBO ; A1KHV0, PHS_MYCBP ; DR C1AMF4, PHS_MYCBT ; B8ZRQ6, PHS_MYCLB ; Q9CBX1, PHS_MYCLE ; DR P61733, PHS_MYCPA ; A0R2K7, PHS_MYCS2 ; A3Q4G7, PHS_MYCSJ ; DR A1UKF3, PHS_MYCSK ; Q1B4N5, PHS_MYCSS ; A5U1K8, PHS_MYCTA ; DR P9WI92, PHS_MYCTO ; P9WI93, PHS_MYCTU ; A1TDS4, PHS_MYCVP ; DR Q3ISB3, PHS_NATPD ; Q0ADS7, PHS_NITEC ; Q82Y13, PHS_NITEU ; DR Q1QHE0, PHS_NITHX ; Q2YB52, PHS_NITMU ; Q3J910, PHS_NITOC ; DR Q3SNS9, PHS_NITWN ; Q5YQC8, PHS_NOCFA ; B2IVV2, PHS_NOSP7 ; DR Q8YNL6, PHS_NOSS1 ; Q2G9B0, PHS_NOVAD ; B1ZY08, PHS_OPITP ; DR A1TND7, PHS_PARC0 ; A1B3I3, PHS_PARDP ; A7HSX5, PHS_PARL1 ; DR Q7U5B3, PHS_PARMW ; B2T7M8, PHS_PARPJ ; Q13SM2, PHS_PARXL ; DR B4SAU0, PHS_PELPB ; B4RBN1, PHS_PHEZH ; Q6LI45, PHS_PHOPR ; DR B1XN71, PHS_PICP2 ; A3PBL6, PHS_PROM0 ; A8G3K7, PHS_PROM2 ; DR A2C7K4, PHS_PROM3 ; A2BVF3, PHS_PROM5 ; Q31C42, PHS_PROM9 ; DR Q7VD93, PHS_PROMA ; Q7V689, PHS_PROMM ; Q7V2I5, PHS_PROMP ; DR A2BPX4, PHS_PROMS ; Q46GT1, PHS_PROMT ; Q48G05, PHS_PSE14 ; DR A6VAA4, PHS_PSEA7 ; B7UY63, PHS_PSEA8 ; Q02I31, PHS_PSEAB ; DR P43335, PHS_PSEAE ; Q1I6Y2, PHS_PSEE4 ; Q4KG97, PHS_PSEF5 ; DR C3K0W5, PHS_PSEFS ; A5W0C1, PHS_PSEP1 ; Q3KG65, PHS_PSEPF ; DR B0KRW3, PHS_PSEPG ; Q88EH2, PHS_PSEPK ; B1JCI6, PHS_PSEPW ; DR Q885L1, PHS_PSESM ; Q3IIZ3, PHS_PSET1 ; Q4ZQG4, PHS_PSEU2 ; DR Q4FVF3, PHS_PSYA2 ; A1SSU3, PHS_PSYIN ; Q8XU38, PHS_RALN1 ; DR B2UGY6, PHS_RALPJ ; P61459, PHS_RAT ; Q2K3A7, PHS_RHIEC ; DR Q1MAM8, PHS_RHIJ3 ; Q98E92, PHS_RHILO ; B5ZTF5, PHS_RHILW ; DR Q92L66, PHS_RHIME ; B9JCJ2, PHS_RHIR8 ; A5V667, PHS_RHIWR ; DR Q2J3Z9, PHS_RHOP2 ; Q07N39, PHS_RHOP5 ; P61734, PHS_RHOPA ; DR Q214W1, PHS_RHOPB ; Q13D98, PHS_RHOPS ; B3Q9E4, PHS_RHOPT ; DR Q2RNC0, PHS_RHORT ; A8GYB5, PHS_RICB8 ; Q1RGM6, PHS_RICBR ; DR A8EXY4, PHS_RICCK ; Q4UKS5, PHS_RICFE ; A7NS25, PHS_ROSCS ; DR Q16BN1, PHS_ROSDO ; A5UPS5, PHS_ROSS1 ; Q1AUE0, PHS_RUBXD ; DR Q5LM31, PHS_RUEPO ; Q1GDR0, PHS_RUEST ; Q21MC6, PHS_SACD2 ; DR Q97WM6, PHS_SACS2 ; O42658, PHS_SCHPO ; A1S7S0, PHS_SHEAM ; DR B8E7N1, PHS_SHEB2 ; A3D2P0, PHS_SHEB5 ; A6WLE1, PHS_SHEB8 ; DR A9KVE9, PHS_SHEB9 ; Q12L03, PHS_SHEDO ; Q084Y3, PHS_SHEFN ; DR B0TNB2, PHS_SHEHH ; A3QCV5, PHS_SHELP ; Q8EGD7, PHS_SHEON ; DR A8H2I7, PHS_SHEPA ; A4Y581, PHS_SHEPC ; B8CLA1, PHS_SHEPW ; DR A0KYY8, PHS_SHESA ; Q0HGZ0, PHS_SHESM ; Q0HT97, PHS_SHESR ; DR A1RLI8, PHS_SHESW ; B1KMK3, PHS_SHEWM ; C3MAI8, PHS_SINFN ; DR A6UE17, PHS_SINMW ; Q01VX3, PHS_SOLUE ; Q82M02, PHS_STRAW ; DR O86722, PHS_STRCO ; B4SPV4, PHS_STRM5 ; B2FM88, PHS_STRMK ; DR A4VQE0, PHS_STUS1 ; Q4J6J6, PHS_SULAC ; C3MZU9, PHS_SULIA ; DR C4KJC1, PHS_SULIK ; C3MTL3, PHS_SULIM ; Q96YL0, PHS_SULTO ; DR Q3A282, PHS_SYNC1 ; A5GMR6, PHS_SYNPW ; Q0I8H5, PHS_SYNS3 ; DR P73790, PHS_SYNY3 ; B9L1Y8, PHS_THERP ; Q8DHW8, PHS_THEVB ; DR Q3SHT9, PHS_THIDA ; Q116J2, PHS_TRIEI ; Q3MA60, PHS_TRIV2 ; DR A7N3S9, PHS_VIBC1 ; A5F0J0, PHS_VIBC3 ; Q9KLB9, PHS_VIBCH ; DR C3LW91, PHS_VIBCM ; Q87IM9, PHS_VIBPA ; Q8D6S1, PHS_VIBVU ; DR Q7MDN2, PHS_VIBVY ; Q8PKQ3, PHS_XANAC ; Q3BSV4, PHS_XANC5 ; DR Q4UUW5, PHS_XANC8 ; B0RTH5, PHS_XANCB ; Q8P8Z8, PHS_XANCP ; DR Q2P1A2, PHS_XANOM ; B2STN6, PHS_XANOP ; Q5GYA0, PHS_XANOR ; DR Q91901, PHS_XENLA ; B2I9W6, PHS_XYLF2 ; Q9PAB4, PHS_XYLFA ; DR B0U5V4, PHS_XYLFM ; Q87A51, PHS_XYLFT ; P38744, PHS_YEAST ; //