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ENZYME

ENZYME entry: EC 4.2.1.96

Accepted Name
4a-hydroxytetrahydrobiopterin dehydratase
Alternative Name(s)
4alpha-hydroxy-tetrahydropterin dehydratase
pterin-4alpha-carbinolamine dehydratase
tetrahydrobiopterin dehydratase
Reaction catalysed
(4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin <=> (6R)-L-erythro-6,7-dihydrobiopterin + H2O
Comment(s)
  • In concert with EC 1.5.1.34 the enzyme recycles 4a-hydroxytetrahydrobiopterin back to tetrahydrobiopterin, a cosubstrate for several enzymes, including aromatic amino acid hydroxylases.
  • The enzyme is bifunctional, and also acts as a dimerization cofactor of hepatocyte nuclear factor-1alpha (HNF-1).
Cross-references
BRENDA4.2.1.96
EC2PDB4.2.1.96
ExplorEnz4.2.1.96
PRIAM enzyme-specific profiles4.2.1.96
KEGG Ligand Database for Enzyme Nomenclature4.2.1.96
IUBMB Enzyme Nomenclature4.2.1.96
IntEnz4.2.1.96
MEDLINEFind literature relating to 4.2.1.96
MetaCyc4.2.1.96
Rhea expert-curated reactions4.2.1.96
UniProtKB/Swiss-Prot
Q9LU63, ATP1_ARATHQ6QJ72, PDL2_ARATHQ475Y4, PHS1_CUPPJ
Q7NM43, PHS1_GLOVIQ9DG45, PHS2_CHICKQ46VT8, PHS2_CUPPJ
Q7NK35, PHS2_GLOVIQ9H0N5, PHS2_HUMANQ9CZL5, PHS2_MOUSE
Q5R7K1, PHS2_PONABA1TND7, PHS_ACIACC1F425, PHS_ACIC5
A5FWK8, PHS_ACICJA0KJG4, PHS_AERHHA4SNJ1, PHS_AERS4
B6JAT9, PHS_AFIC5Q8UC22, PHS_AGRFCB9JCJ2, PHS_AGRRK
B9JUA0, PHS_AGRVSA6TVX6, PHS_ALKMQB4RXT2, PHS_ALTMD
O66462, PHS_AQUAEQ5P905, PHS_AROAEA1K7V8, PHS_AZOSB
C1DQH9, PHS_AZOVDP61732, PHS_BDEBAQ2KXB6, PHS_BORA1
Q7WG14, PHS_BORBRQ7W4J0, PHS_BORPAQ7VU17, PHS_BORPE
Q3ZBD3, PHS_BOVINQ89WZ6, PHS_BRADUA5E8P9, PHS_BRASB
A4YJY8, PHS_BRASOB2S7X9, PHS_BRUA1Q2YNV0, PHS_BRUA2
A6WV21, PHS_BRUA4Q57FT6, PHS_BRUABA9M6T4, PHS_BRUC2
C0RGE2, PHS_BRUMBP65722, PHS_BRUMEA5VN26, PHS_BRUO2
B0CIJ4, PHS_BRUSIP65723, PHS_BRUSUB1YQ64, PHS_BURA4
A0K2V4, PHS_BURCHB4E566, PHS_BURCJQ0BJP4, PHS_BURCM
Q39L06, PHS_BURL3A9AJD5, PHS_BURM1Q62FU4, PHS_BURMA
B1K1I7, PHS_BURO0Q3JXT0, PHS_BURP1Q63PF1, PHS_BURPS
A4JA07, PHS_BURVGQ9TZH6, PHS_CAEELB0T207, PHS_CAUSK
Q9ABI2, PHS_CAUVCB8GYB5, PHS_CAUVNQ3IZF4, PHS_CERS4
Q11D07, PHS_CHESBO73930, PHS_CHICKA9WI69, PHS_CHLAA
B8G3F7, PHS_CHLADB3ECB9, PHS_CHLL2Q3B6N5, PHS_CHLL3
B3QQ83, PHS_CHLP8B9LCC8, PHS_CHLSYQ8KFI4, PHS_CHLTE
Q1QXY2, PHS_CHRSDQ7NVH7, PHS_CHRVOQ47XN8, PHS_COLP3
B8HU56, PHS_CYAP4Q11PN9, PHS_CYTH3Q47IX1, PHS_DECAR
Q54RY8, PHS_DICDIA8LKP0, PHS_DINSHO76454, PHS_DROME
P58249, PHS_DROVIA0M1H4, PHS_GRAFKQ2SCE8, PHS_HAHCH
A1WWH9, PHS_HALHLB9LPE3, PHS_HALLTQ5V1I9, PHS_HALMA
B0R6B9, PHS_HALS3Q9HP73, PHS_HALSAA9AXS2, PHS_HERA2
P61457, PHS_HUMANQ31HZ6, PHS_HYDCUP0C8L6, PHS_HYPEX
Q5QU19, PHS_IDILOQ28VA7, PHS_JANSCQ1IP68, PHS_KORVE
Q5X3Q4, PHS_LEGPAA5IDI1, PHS_LEGPCQ5ZU09, PHS_LEGPH
Q5WV42, PHS_LEGPLA6VSD2, PHS_MARMSA1TZA3, PHS_MARN8
Q60BH8, PHS_METCAA4YEM3, PHS_METS5B0JQV0, PHS_MICAN
P61458, PHS_MOUSEA0QCA6, PHS_MYCA1B1MLG8, PHS_MYCA9
P0A5S3, PHS_MYCBOA1KHV0, PHS_MYCBPC1AMF4, PHS_MYCBT
B8ZRQ6, PHS_MYCLBQ9CBX1, PHS_MYCLEP61733, PHS_MYCPA
A0R2K7, PHS_MYCS2A3Q4G7, PHS_MYCSJA1UKF3, PHS_MYCSK
Q1B4N5, PHS_MYCSSA5U1K8, PHS_MYCTAP9WI92, PHS_MYCTO
P9WI93, PHS_MYCTUA1TDS4, PHS_MYCVPQ3ISB3, PHS_NATPD
Q0ADS7, PHS_NITECQ82Y13, PHS_NITEUQ1QHE0, PHS_NITHX
Q2YB52, PHS_NITMUQ3J910, PHS_NITOCQ3SNS9, PHS_NITWN
Q5YQC8, PHS_NOCFAB2IVV2, PHS_NOSP7Q8YNL6, PHS_NOSS1
Q2G9B0, PHS_NOVADB1ZY08, PHS_OPITPA1B3I3, PHS_PARDP
A7HSX5, PHS_PARL1Q7U5B3, PHS_PARMWB2T7M8, PHS_PARPJ
Q13SM2, PHS_PARXLB4SAU0, PHS_PELPBB4RBN1, PHS_PHEZH
Q6LI45, PHS_PHOPRA3PBL6, PHS_PROM0A8G3K7, PHS_PROM2
A2C7K4, PHS_PROM3A2BVF3, PHS_PROM5Q31C42, PHS_PROM9
Q7VD93, PHS_PROMAQ7V689, PHS_PROMMQ7V2I5, PHS_PROMP
A2BPX4, PHS_PROMSQ46GT1, PHS_PROMTQ48G05, PHS_PSE14
A6VAA4, PHS_PSEA7B7UY63, PHS_PSEA8Q02I31, PHS_PSEAB
P43335, PHS_PSEAEQ1I6Y2, PHS_PSEE4Q4KG97, PHS_PSEF5
C3K0W5, PHS_PSEFSA5W0C1, PHS_PSEP1Q3KG65, PHS_PSEPF
B0KRW3, PHS_PSEPGQ88EH2, PHS_PSEPKB1JCI6, PHS_PSEPW
Q885L1, PHS_PSESMQ3IIZ3, PHS_PSET1Q4ZQG4, PHS_PSEU2
Q4FVF3, PHS_PSYA2A1SSU3, PHS_PSYINB2UGY6, PHS_RALPJ
Q8XU38, PHS_RALSOP61459, PHS_RATQ2K3A7, PHS_RHIEC
Q1MAM8, PHS_RHIL3Q98E92, PHS_RHILOB5ZTF5, PHS_RHILW
Q92L66, PHS_RHIMEA5V667, PHS_RHIWRQ2J3Z9, PHS_RHOP2
Q07N39, PHS_RHOP5P61734, PHS_RHOPAQ214W1, PHS_RHOPB
Q13D98, PHS_RHOPSB3Q9E4, PHS_RHOPTQ2RNC0, PHS_RHORT
A8GYB5, PHS_RICB8Q1RGM6, PHS_RICBRA8EXY4, PHS_RICCK
Q4UKS5, PHS_RICFEA7NS25, PHS_ROSCSQ16BN1, PHS_ROSDO
A5UPS5, PHS_ROSS1Q1AUE0, PHS_RUBXDQ5LM31, PHS_RUEPO
Q1GDR0, PHS_RUESTQ21MC6, PHS_SACD2Q97WM6, PHS_SACS2
O42658, PHS_SCHPOA1S7S0, PHS_SHEAMB8E7N1, PHS_SHEB2
A3D2P0, PHS_SHEB5A6WLE1, PHS_SHEB8A9KVE9, PHS_SHEB9
Q12L03, PHS_SHEDOQ084Y3, PHS_SHEFNB0TNB2, PHS_SHEHH
A3QCV5, PHS_SHELPQ8EGD7, PHS_SHEONA8H2I7, PHS_SHEPA
A4Y581, PHS_SHEPCB8CLA1, PHS_SHEPWA0KYY8, PHS_SHESA
Q0HGZ0, PHS_SHESMQ0HT97, PHS_SHESRA1RLI8, PHS_SHESW
B1KMK3, PHS_SHEWMC3MAI8, PHS_SINFNA6UE17, PHS_SINMW
Q01VX3, PHS_SOLUEQ82M02, PHS_STRAWO86722, PHS_STRCO
B4SPV4, PHS_STRM5B2FM88, PHS_STRMKA4VQE0, PHS_STUS1
Q4J6J6, PHS_SULACC3MZU9, PHS_SULIAC4KJC1, PHS_SULIK
C3MTL3, PHS_SULIMQ96YL0, PHS_SULTOQ3A282, PHS_SYNC1
B1XN71, PHS_SYNP2A5GMR6, PHS_SYNPWQ0I8H5, PHS_SYNS3
P73790, PHS_SYNY3B9L1Y8, PHS_THERPQ8DHW8, PHS_THEVB
Q3SHT9, PHS_THIDAQ116J2, PHS_TRIEIQ3MA60, PHS_TRIV2
A7N3S9, PHS_VIBC1A5F0J0, PHS_VIBC3Q9KLB9, PHS_VIBCH
C3LW91, PHS_VIBCMQ87IM9, PHS_VIBPAQ8D6S1, PHS_VIBVU
Q7MDN2, PHS_VIBVYQ8PKQ3, PHS_XANACQ3BSV4, PHS_XANC5
Q4UUW5, PHS_XANC8B0RTH5, PHS_XANCBQ8P8Z8, PHS_XANCP
Q2P1A2, PHS_XANOMB2STN6, PHS_XANOPQ5GYA0, PHS_XANOR
Q91901, PHS_XENLAB2I9W6, PHS_XYLF2Q9PAB4, PHS_XYLFA
B0U5V4, PHS_XYLFMQ87A51, PHS_XYLFTP38744, PHS_YEAST

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All UniProtKB/Swiss-Prot entries referenced in this entry, with possibility to download in different formats, align etc.
All ENZYME / UniProtKB/Swiss-Prot entries corresponding to 4.2.1.-
All ENZYME / UniProtKB/Swiss-Prot entries corresponding to 4.2.-.-
All ENZYME / UniProtKB/Swiss-Prot entries corresponding to 4.-.-.-