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ENZYME

ENZYME entry: EC 2.5.1.61

Accepted Name
hydroxymethylbilane synthase
Alternative Name(s)
(4-[2-carboxyethyl]-3-[carboxymethyl]pyrrol-2-yl)methyltransferase (hydrolyzing)
HMB-synthase
porphobilinogen deaminase
pre-uroporphyrinogen synthase
uroporphyrinogen I synthase
uroporphyrinogen I synthetase
uroporphyrinogen synthase
uroporphyrinogen synthetase
Reaction catalysed
H2O + 4 porphobilinogen = hydroxymethylbilane + 4 NH4(+)
Comment(s)
  • The enzyme works by stepwise addition of pyrrolylmethyl groups until a hexapyrrole is present at the active center.
  • The terminal tetrapyrrole is then hydrolyzed to yield the product, leaving a cysteine-bound dipyrrole on which assembly continues.
  • In the presence of a second enzyme, EC 4.2.1.75, which is often called cosynthase, the product is cyclized to form uroporphyrinogen III.
  • If EC 4.2.1.75 is absent, the hydroxymethylbilane cyclizes spontaneously to form uroporphyrinogen I.
  • Formerly EC 4.3.1.8.
Cross-references
BRENDA2.5.1.61
EC2PDB2.5.1.61
ExplorEnz2.5.1.61
PRIAM enzyme-specific profiles2.5.1.61
KEGG Ligand Database for Enzyme Nomenclature2.5.1.61
IUBMB Enzyme Nomenclature2.5.1.61
IntEnz2.5.1.61
MEDLINEFind literature relating to 2.5.1.61
MetaCyc2.5.1.61
Rhea expert-curated reactions2.5.1.61
UniProtKB/Swiss-Prot
Q82E76, HEM31_STRAWQ9WX16, HEM31_STRCOQ82P95, HEM32_STRAW
Q9KY00, HEM32_STRCOA3DIF0, HEM3_ACET2Q6FFA9, HEM3_ACIAD
A0LRF1, HEM3_ACIC1B7JBH8, HEM3_ACIF2B5ERN6, HEM3_ACIF5
A6VL87, HEM3_ACTSZQ9Y9J0, HEM3_AERPEQ8UC46, HEM3_AGRFC
Q21XQ9, HEM3_ALBFTQ0VM28, HEM3_ALCBSA8ERI4, HEM3_ALIB4
Q5E8T5, HEM3_ALIF1B5FF97, HEM3_ALIFMB6EPK1, HEM3_ALISL
Q0A577, HEM3_ALKEHA6TJD5, HEM3_ALKMQA8MGE4, HEM3_ALKOO
O66621, HEM3_AQUAEQ43316, HEM3_ARATHO29026, HEM3_ARCFU
C3P9E9, HEM3_BACAAC3L6Z5, HEM3_BACACA0RJ81, HEM3_BACAH
Q81LC7, HEM3_BACANB7JQ57, HEM3_BACC0Q72ZW2, HEM3_BACC1
B7IIX5, HEM3_BACC2C1ETR0, HEM3_BACC3B7HE96, HEM3_BACC4
B7HQM4, HEM3_BACC7A7GTE5, HEM3_BACCNB9IZ39, HEM3_BACCQ
Q817R0, HEM3_BACCRQ633Y0, HEM3_BACCZQ6HD62, HEM3_BACHK
Q65GK1, HEM3_BACLDA9VIT8, HEM3_BACMKA8FFV2, HEM3_BACP2
P16616, HEM3_BACSUA7Z7A5, HEM3_BACVZQ1LU25, HEM3_BAUCH
Q7VRM4, HEM3_BLOFLQ491Z6, HEM3_BLOPBQ7WKM1, HEM3_BORBR
Q7W785, HEM3_BORPAA9IKF3, HEM3_BORPDQ7VVU9, HEM3_BORPE
Q2KIN5, HEM3_BOVINC0ZAH1, HEM3_BREBNB2S842, HEM3_BRUA1
Q2YLM5, HEM3_BRUA2A6WXJ2, HEM3_BRUA4Q57B08, HEM3_BRUAB
A9M8M2, HEM3_BRUC2C0RFD1, HEM3_BRUMBQ8YJB0, HEM3_BRUME
A5VSL5, HEM3_BRUO2B0CID9, HEM3_BRUSIQ8FYI6, HEM3_BRUSU
B8D8F3, HEM3_BUCA5P57651, HEM3_BUCAIB8D8B0, HEM3_BUCAT
B4E8C4, HEM3_BURCJQ0BCU9, HEM3_BURCMA3MLK5, HEM3_BURM7
A2SAH5, HEM3_BURM9Q62LC0, HEM3_BURMAA1V5U0, HEM3_BURMS
B1JWR0, HEM3_BURO0A3NSN6, HEM3_BURP0Q3JUW3, HEM3_BURP1
A3N700, HEM3_BURP6Q63W73, HEM3_BURPSQ2T071, HEM3_BURTA
A4JGV4, HEM3_BURVGA8MDU3, HEM3_CALMQA4XK06, HEM3_CALS8
A7ZD37, HEM3_CAMC1A7GYU4, HEM3_CAMC5A0RNT3, HEM3_CAMFF
A7I1S0, HEM3_CAMHCA8FKW8, HEM3_CAMJ8A7H4J7, HEM3_CAMJD
Q9PHW9, HEM3_CAMJEA1VYQ4, HEM3_CAMJJQ5HVM1, HEM3_CAMJR
B9KGA9, HEM3_CAMLRO94048, HEM3_CANALQ6FNR4, HEM3_CANGA
Q3ACT3, HEM3_CARHZB0T287, HEM3_CAUSKQ9ABZ8, HEM3_CAUVC
B8GXG1, HEM3_CAUVNQ3AQF5, HEM3_CHLCHB3EIN1, HEM3_CHLL2
Q3B2Z5, HEM3_CHLL3Q9PK95, HEM3_CHLMUP28464, HEM3_CHLP8
B3EPK0, HEM3_CHLPBA1BHD6, HEM3_CHLPDA4SFJ8, HEM3_CHLPM
Q9Z9C9, HEM3_CHLPNB3QWI1, HEM3_CHLT3Q8KCJ4, HEM3_CHLTE
O84301, HEM3_CHLTRQ7P207, HEM3_CHRVOB5EBG8, HEM3_CITBB
Q97MU4, HEM3_CLOABA7FSG8, HEM3_CLOB1C3L2Z3, HEM3_CLOB6
A6LSY5, HEM3_CLOB8B2UXC4, HEM3_CLOBAB2TPD7, HEM3_CLOBB
A5I0B2, HEM3_CLOBHC1FVB9, HEM3_CLOBJB1IHD1, HEM3_CLOBK
A7GBW3, HEM3_CLOBLB1KY15, HEM3_CLOBMQ180R9, HEM3_CLOD6
B9DZL6, HEM3_CLOK1A0Q2B1, HEM3_CLONNQ0TQG4, HEM3_CLOP1
Q8XKG4, HEM3_CLOPEQ0ST16, HEM3_CLOPSQ48AW8, HEM3_COLP3
C3PKG2, HEM3_CORA7Q6NJK0, HEM3_CORDIQ8FSF7, HEM3_COREF
A4QB59, HEM3_CORGBQ8NT90, HEM3_CORGLB6J6K0, HEM3_COXB1
B6J3T6, HEM3_COXB2A9KDC4, HEM3_COXBNA9N910, HEM3_COXBR
Q83A37, HEM3_COXBUA7MQJ1, HEM3_CROS8Q6AB05, HEM3_CUTAK
Q11XT5, HEM3_CYTH3Q6BM23, HEM3_DEBHAQ479S8, HEM3_DECAR
C1CYD9, HEM3_DEIDVQ1J288, HEM3_DEIGDQ9RRY0, HEM3_DEIRA
B1I4L8, HEM3_DESAPB8G2M2, HEM3_DESHDQ24VC8, HEM3_DESHY
A8ZWG5, HEM3_DESOHA4J6H7, HEM3_DESRMP40129, HEM3_DICCH
Q54P93, HEM3_DICDIA5EW83, HEM3_DICNVB7MH66, HEM3_ECO45
B7L959, HEM3_ECO55Q8XAP3, HEM3_ECO57B7NTE0, HEM3_ECO7I
B7N295, HEM3_ECO81B7M603, HEM3_ECO8AC4ZZ69, HEM3_ECOBW
B1XAH2, HEM3_ECODHQ8FBP1, HEM3_ECOL6P06983, HEM3_ECOLI
B7NFA7, HEM3_ECOLUB6I4E0, HEM3_ECOSEQ3YSA8, HEM3_EHRCJ
Q2GGC7, HEM3_EHRCRA4WG12, HEM3_ENT38Q75DY0, HEM3_EREGS
B2VG55, HEM3_ERWT9B7LU53, HEM3_ESCF3P13446, HEM3_EUGGR
B1YJV1, HEM3_EXIS2C4L4J6, HEM3_EXISAB0TW04, HEM3_FRAP2
Q14JI3, HEM3_FRAT1A7N9H6, HEM3_FRATFQ2A5R3, HEM3_FRATH
B2SE34, HEM3_FRATMA0Q481, HEM3_FRATNQ0BP15, HEM3_FRATO
Q5NI31, HEM3_FRATTA4IZY4, HEM3_FRATWQ8RFP5, HEM3_FUSNN
B9M416, HEM3_GEODFQ5KWK6, HEM3_GEOKAQ39QM7, HEM3_GEOMG
Q747I1, HEM3_GEOSLC6DYY8, HEM3_GEOSMC5D5K7, HEM3_GEOSW
A4IRG5, HEM3_GEOTNA5GCW2, HEM3_GEOURQ7NGF7, HEM3_GLOVI
Q2SQ70, HEM3_HAHCHQ9K8G0, HEM3_HALH5A1WVT9, HEM3_HALHL
Q9HMY5, HEM3_HALSAC4K4E3, HEM3_HAMD5Q17W61, HEM3_HELAH
Q7VFE9, HEM3_HELHPB6JKG8, HEM3_HELP2B5ZA04, HEM3_HELPG
Q1CUR5, HEM3_HELPHQ9ZMJ7, HEM3_HELPJB2US69, HEM3_HELPS
P56140, HEM3_HELPYQ0I178, HEM3_HISS1B0UWZ8, HEM3_HISS2
P08397, HEM3_HUMANQ31JG5, HEM3_HYDCUA2BL26, HEM3_HYPBU
Q0BX92, HEM3_HYPNAQ5QUS3, HEM3_IDILOA8AAB4, HEM3_IGNH4
B5XYL1, HEM3_KLEP3A6TGI8, HEM3_KLEP7Q6CT60, HEM3_KLULA
Q1INI6, HEM3_KORVEC4Z552, HEM3_LACE2C1D679, HEM3_LARHH
Q1MPL2, HEM3_LAWIPQ5X1F2, HEM3_LEGPAQ5ZRY6, HEM3_LEGPH
Q5WT65, HEM3_LEGPLQ6AHF1, HEM3_LEIXXA5VM67, HEM3_LIMRD
B2G9H4, HEM3_LIMRJQ92BF8, HEM3_LISINC1KVK3, HEM3_LISMC
Q71ZB2, HEM3_LISMFB8DHJ2, HEM3_LISMHQ8Y6X5, HEM3_LISMO
A0AJ05, HEM3_LISW6B1HVD6, HEM3_LYSSCB9E743, HEM3_MACCJ
Q65VX7, HEM3_MANSMA1TXW9, HEM3_MARN8C6BYB7, HEM3_MARSD
Q8TT56, HEM3_METACQ0W5T2, HEM3_METARQ46CH0, HEM3_METBF
Q12WM8, HEM3_METBUQ602K3, HEM3_METCAQ1GXA1, HEM3_METFK
Q57989, HEM3_METJAQ8TXC8, HEM3_METKAQ8PW57, HEM3_METMA
A4YD92, HEM3_METS5O26960, HEM3_METTHC5CB88, HEM3_MICLC
Q2RJ26, HEM3_MOOTAP22907, HEM3_MOUSEA0QLI0, HEM3_MYCA1
B1MHI5, HEM3_MYCA9P64337, HEM3_MYCBOA1KFY6, HEM3_MYCBP
C1AKJ0, HEM3_MYCBTA4T340, HEM3_MYCGIQ49808, HEM3_MYCLE
B2HRJ2, HEM3_MYCMMQ73SS0, HEM3_MYCPAA5TZP0, HEM3_MYCTA
P9WMP2, HEM3_MYCTOP9WMP3, HEM3_MYCTUA0PW29, HEM3_MYCUA
A1T3D3, HEM3_MYCVPQ1D8Z8, HEM3_MYXXDB2A1G7, HEM3_NATTJ
Q9JVS4, HEM3_NEIMAQ9K0P6, HEM3_NEIMBQ6MW51, HEM3_NEUCR
Q82WS2, HEM3_NITEUQ1QQP5, HEM3_NITHXA9A1A1, HEM3_NITMS
A6Q3S1, HEM3_NITSBQ72AV0, HEM3_NITV2A1VCX6, HEM3_NITV4
B8DKW2, HEM3_NITV9Q3SVF3, HEM3_NITWNQ5YP70, HEM3_NOCFA
Q8YVU6, HEM3_NOSS1Q8CXC0, HEM3_OCEIHQ30Y29, HEM3_OLEA2
A5CDH4, HEM3_ORITBB3CUP9, HEM3_ORITIQ6H6D2, HEM3_ORYSJ
O69110, HEM3_PAEMAA1B853, HEM3_PARDPQ7U5C2, HEM3_PARMW
B2JEN9, HEM3_PARP8B2T1D1, HEM3_PARPJQ9CK24, HEM3_PASMU
Q43082, HEM3_PEAQ6CZG3, HEM3_PECASC6DHC2, HEM3_PECCP
B4SFI2, HEM3_PELPBA1AUE7, HEM3_PELPDA5D3L5, HEM3_PELTS
B4RC21, HEM3_PHEZHQ7MYN1, HEM3_PHOLLQ6L2G8, HEM3_PICTO
B4S922, HEM3_PROA2A3PBM0, HEM3_PROM0A2C0V4, HEM3_PROM1
A8G3L1, HEM3_PROM2A2C7L8, HEM3_PROM3A9BEB6, HEM3_PROM4
A2BVF7, HEM3_PROM5Q31C38, HEM3_PROM9Q7VD89, HEM3_PROMA
B4F1X3, HEM3_PROMHQ59684, HEM3_PROMIQ7V697, HEM3_PROMM
Q7V2I1, HEM3_PROMPA2BPX7, HEM3_PROMSQ46GS7, HEM3_PROMT
Q15Z15, HEM3_PSEA6A6VE37, HEM3_PSEA7B7V5F0, HEM3_PSEA8
Q02EA3, HEM3_PSEABQ60169, HEM3_PSEAEQ1I316, HEM3_PSEE4
Q4K3X3, HEM3_PSEF5C3K424, HEM3_PSEFSA5VWX3, HEM3_PSEP1
Q3K4T0, HEM3_PSEPFB0KH05, HEM3_PSEPGQ88RE5, HEM3_PSEPK
B1J1V2, HEM3_PSEPWQ88B91, HEM3_PSESMQ500N6, HEM3_PSEU2
A1T0R0, HEM3_PSYINQ8ZYW7, HEM3_PYRAEA4WN24, HEM3_PYRAR
A3MWV7, HEM3_PYRCJA1RQK2, HEM3_PYRILB1YDP0, HEM3_PYRNV
Q8XWW3, HEM3_RALN1P19356, HEM3_RATQ98EI7, HEM3_RHILO
Q92LH7, HEM3_RHIMEQ7UPN0, HEM3_RHOBAC3PNS9, HEM3_RICAE
A8GNR5, HEM3_RICAHA8GXC8, HEM3_RICB8Q1RJN7, HEM3_RICBR
A8EYJ9, HEM3_RICCKQ92HR5, HEM3_RICCNQ4ULA5, HEM3_RICFE
A8F1Y8, HEM3_RICM5Q9ZD77, HEM3_RICPRC4K265, HEM3_RICPU
B0BY00, HEM3_RICROA8GSI6, HEM3_RICRSQ68WR3, HEM3_RICTY
A7NKU8, HEM3_ROSCSA5UU37, HEM3_ROSS1Q1AUK3, HEM3_RUBXD
B8I0Q8, HEM3_RUMCHQ59293, HEM3_RUMJOA1AX51, HEM3_RUTMC
Q980U4, HEM3_SACS2A9MJ10, HEM3_SALARB5FN93, HEM3_SALDC
Q5PKL7, HEM3_SALPAC0Q3B3, HEM3_SALPCB5BIU8, HEM3_SALPK
B4TNV3, HEM3_SALSVP0A1Q9, HEM3_SALTIP0A1Q8, HEM3_SALTY
Q09899, HEM3_SCHPOA8G848, HEM3_SERP5B8E879, HEM3_SHEB2
A3D9Q4, HEM3_SHEB5A6WTG0, HEM3_SHEB8A9L5G0, HEM3_SHEB9
Q088L3, HEM3_SHEFNB0TJ54, HEM3_SHEHHQ8E9H0, HEM3_SHEON
A8GZG4, HEM3_SHEPAA4YBG2, HEM3_SHEPCA0KS60, HEM3_SHESA
A8G0W4, HEM3_SHESHQ0HN99, HEM3_SHESMQ0HQI7, HEM3_SHESR
A1RPD7, HEM3_SHESWB1KQC6, HEM3_SHEWMQ83PH4, HEM3_SHIFL
Q5WEP5, HEM3_SHOC1A9FDP9, HEM3_SORC5Q1GP41, HEM3_SPHAL
A7X391, HEM3_STAA1A6U2D8, HEM3_STAA2Q2FG66, HEM3_STAA3
O34090, HEM3_STAA8A5ITJ5, HEM3_STAA9Q2YTB9, HEM3_STAAB
Q5HFA2, HEM3_STAACA6QHK4, HEM3_STAAEP64340, HEM3_STAAM
P64341, HEM3_STAANQ6GG35, HEM3_STAARQ6G8Q5, HEM3_STAAS
A8Z2J1, HEM3_STAATQ8NW74, HEM3_STAAWB9DNE1, HEM3_STACT
Q5HNN3, HEM3_STAEQQ8CNY8, HEM3_STAESQ4L709, HEM3_STAHJ
Q49YA2, HEM3_STAS1B4SJT8, HEM3_STRM5B2FI06, HEM3_STRMK
A3CL78, HEM3_STRSVQ30S90, HEM3_SULDNC3MZR8, HEM3_SULIA
C4KIZ9, HEM3_SULIKC3MJ21, HEM3_SULILC3MYD9, HEM3_SULIM
C3NF70, HEM3_SULINC3N841, HEM3_SULIYA6Q7Y4, HEM3_SULNB
Q976H1, HEM3_SULTOQ2LQU2, HEM3_SYNASQ3A009, HEM3_SYNC1
Q31PM2, HEM3_SYNE7A0LN73, HEM3_SYNFMA5GSE0, HEM3_SYNR3
Q0I8I3, HEM3_SYNS3Q3AWQ4, HEM3_SYNS9Q3ALS9, HEM3_SYNSC
Q0AZ43, HEM3_SYNWWP73660, HEM3_SYNY3Q9HKM5, HEM3_THEAC
Q47LA7, HEM3_THEFYQ72H57, HEM3_THET2Q5SLE8, HEM3_THET8
Q8DIE4, HEM3_THEVBQ97B26, HEM3_THEVOQ3SFT5, HEM3_THIDA
B8GSU8, HEM3_THISHB3E2H7, HEM3_TRIL1A5CW34, HEM3_VESOH
B7VMD9, HEM3_VIBA3A7MXU9, HEM3_VIBC1Q9KVM1, HEM3_VIBCH
Q87KI9, HEM3_VIBPAQ8DD85, HEM3_VIBVUQ7MQC7, HEM3_VIBVY
Q7M8L2, HEM3_WOLSUC0R2M3, HEM3_WOLWRQ8PPR3, HEM3_XANAC
Q4UYZ4, HEM3_XANC8B0RNI9, HEM3_XANCBQ8P536, HEM3_XANCP
Q3BXV3, HEM3_XANE5Q2NYU4, HEM3_XANOMB2SMV3, HEM3_XANOP
Q5GVL2, HEM3_XANORB2I5K8, HEM3_XYLF2Q9PCX7, HEM3_XYLFA
B0U2W8, HEM3_XYLFMQ87CC9, HEM3_XYLFTQ6C097, HEM3_YARLI
P28789, HEM3_YEASTA1JI86, HEM3_YERE8P30527, HEM3_YERIN
A7FD67, HEM3_YERP3Q1CBN1, HEM3_YERPAB2K065, HEM3_YERPB
P46355, HEM3_YERPEA9R8K0, HEM3_YERPGQ1CNI3, HEM3_YERPN
A4TRA8, HEM3_YERPPQ66G00, HEM3_YERPSB1JPE6, HEM3_YERPY

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All ENZYME / UniProtKB/Swiss-Prot entries corresponding to 2.5.1.-
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