Expasy logo

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
Q5WEP5, HEM3_ALKCKQ0A577, HEM3_ALKEHA6TJD5, HEM3_ALKMQ
A8MGE4, HEM3_ALKOOO66621, HEM3_AQUAEQ43316, HEM3_ARATH
O29026, HEM3_ARCFUQ75DY0, HEM3_ASHGOC3P9E9, HEM3_BACAA
C3L6Z5, HEM3_BACACA0RJ81, HEM3_BACAHQ81LC7, HEM3_BACAN
B7JQ57, HEM3_BACC0Q72ZW2, HEM3_BACC1B7IIX5, HEM3_BACC2
C1ETR0, HEM3_BACC3B7HE96, HEM3_BACC4B7HQM4, HEM3_BACC7
A7GTE5, HEM3_BACCNB9IZ39, HEM3_BACCQQ817R0, HEM3_BACCR
Q633Y0, HEM3_BACCZQ6HD62, HEM3_BACHKQ65GK1, HEM3_BACLD
A9VIT8, HEM3_BACMKA8FFV2, HEM3_BACP2P16616, HEM3_BACSU
A7Z7A5, HEM3_BACVZQ1LU25, HEM3_BAUCHQ7VRM4, HEM3_BLOFL
Q491Z6, HEM3_BLOPBQ7WKM1, HEM3_BORBRQ7W785, HEM3_BORPA
A9IKF3, HEM3_BORPDQ7VVU9, HEM3_BORPEQ2KIN5, HEM3_BOVIN
C0ZAH1, HEM3_BREBNB2S842, HEM3_BRUA1Q2YLM5, HEM3_BRUA2
A6WXJ2, HEM3_BRUA4Q57B08, HEM3_BRUABA9M8M2, HEM3_BRUC2
C0RFD1, HEM3_BRUMBQ8YJB0, HEM3_BRUMEA5VSL5, HEM3_BRUO2
B0CID9, HEM3_BRUSIQ8FYI6, HEM3_BRUSUB8D8F3, HEM3_BUCA5
P57651, HEM3_BUCAIB8D8B0, HEM3_BUCATB4E8C4, HEM3_BURCJ
Q0BCU9, HEM3_BURCMA3MLK5, HEM3_BURM7A2SAH5, HEM3_BURM9
Q62LC0, HEM3_BURMAA1V5U0, HEM3_BURMSB1JWR0, HEM3_BURO0
A3NSN6, HEM3_BURP0Q3JUW3, HEM3_BURP1A3N700, HEM3_BURP6
Q63W73, HEM3_BURPSQ2T071, HEM3_BURTAA4JGV4, HEM3_BURVG
A8MDU3, HEM3_CALMQA4XK06, HEM3_CALS8A7ZD37, HEM3_CAMC1
A7GYU4, HEM3_CAMC5A0RNT3, HEM3_CAMFFA7I1S0, HEM3_CAMHC
A8FKW8, HEM3_CAMJ8A7H4J7, HEM3_CAMJDQ9PHW9, HEM3_CAMJE
A1VYQ4, HEM3_CAMJJQ5HVM1, HEM3_CAMJRB9KGA9, HEM3_CAMLR
O94048, HEM3_CANALQ6FNR4, HEM3_CANGBQ3ACT3, HEM3_CARHZ
B0T287, HEM3_CAUSKQ9ABZ8, HEM3_CAUVCB8GXG1, HEM3_CAUVN
Q3AQF5, HEM3_CHLCHB3EIN1, HEM3_CHLL2Q3B2Z5, HEM3_CHLL3
Q9PK95, HEM3_CHLMUP28464, HEM3_CHLP8B3EPK0, HEM3_CHLPB
A1BHD6, HEM3_CHLPDA4SFJ8, HEM3_CHLPMQ9Z9C9, HEM3_CHLPN
B3QWI1, HEM3_CHLT3Q8KCJ4, HEM3_CHLTEO84301, HEM3_CHLTR
Q7P207, HEM3_CHRVOB5EBG8, HEM3_CITBBQ97MU4, HEM3_CLOAB
A7FSG8, HEM3_CLOB1C3L2Z3, HEM3_CLOB6A6LSY5, HEM3_CLOB8
B2UXC4, HEM3_CLOBAB2TPD7, HEM3_CLOBBA5I0B2, HEM3_CLOBH
C1FVB9, HEM3_CLOBJB1IHD1, HEM3_CLOBKA7GBW3, HEM3_CLOBL
B1KY15, HEM3_CLOBMQ180R9, HEM3_CLOD6B9DZL6, HEM3_CLOK1
A0Q2B1, HEM3_CLONNQ0TQG4, HEM3_CLOP1Q8XKG4, HEM3_CLOPE
Q0ST16, HEM3_CLOPSQ48AW8, HEM3_COLP3C3PKG2, HEM3_CORA7
Q6NJK0, HEM3_CORDIQ8FSF7, HEM3_COREFA4QB59, HEM3_CORGB
Q8NT90, HEM3_CORGLB6J6K0, HEM3_COXB1B6J3T6, HEM3_COXB2
A9KDC4, HEM3_COXBNA9N910, HEM3_COXBRQ83A37, HEM3_COXBU
A7MQJ1, HEM3_CROS8Q6AB05, HEM3_CUTAKQ11XT5, HEM3_CYTH3
Q6BM23, HEM3_DEBHAQ479S8, HEM3_DECARC1CYD9, HEM3_DEIDV
Q1J288, HEM3_DEIGDQ9RRY0, HEM3_DEIRAB1I4L8, HEM3_DESAP
B8G2M2, HEM3_DESHDQ24VC8, HEM3_DESHYA8ZWG5, HEM3_DESOH
A4J6H7, HEM3_DESRMQ72AV0, HEM3_DESVHB8DKW2, HEM3_DESVM
A1VCX6, HEM3_DESVVP40129, HEM3_DICCHQ54P93, HEM3_DICDI
A5EW83, HEM3_DICNVB7MH66, HEM3_ECO45B7L959, HEM3_ECO55
Q8XAP3, HEM3_ECO57B7NTE0, HEM3_ECO7IB7N295, HEM3_ECO81
B7M603, HEM3_ECO8AC4ZZ69, HEM3_ECOBWB1XAH2, HEM3_ECODH
Q8FBP1, HEM3_ECOL6P06983, HEM3_ECOLIB7NFA7, HEM3_ECOLU
B6I4E0, HEM3_ECOSEQ3YSA8, HEM3_EHRCJQ2GGC7, HEM3_EHRCR
A4WG12, HEM3_ENT38B2VG55, HEM3_ERWT9B7LU53, HEM3_ESCF3
P13446, HEM3_EUGGRB1YJV1, HEM3_EXIS2C4L4J6, HEM3_EXISA
B0TW04, HEM3_FRAP2Q14JI3, HEM3_FRAT1A7N9H6, HEM3_FRATF
Q2A5R3, HEM3_FRATHB2SE34, HEM3_FRATMA0Q481, HEM3_FRATN
Q0BP15, HEM3_FRATOQ5NI31, HEM3_FRATTA4IZY4, HEM3_FRATW
Q8RFP5, HEM3_FUSNNB9M416, HEM3_GEODFQ5KWK6, HEM3_GEOKA
Q39QM7, HEM3_GEOMGQ747I1, HEM3_GEOSLC6DYY8, HEM3_GEOSM
C5D5K7, HEM3_GEOSWA4IRG5, HEM3_GEOTNA5GCW2, HEM3_GEOUR
Q7NGF7, HEM3_GLOVIQ0I178, HEM3_HAES1Q2SQ70, HEM3_HAHCH
Q9K8G0, HEM3_HALH5A1WVT9, HEM3_HALHLQ9HMY5, HEM3_HALSA
C4K4E3, HEM3_HAMD5Q17W61, HEM3_HELAHQ7VFE9, HEM3_HELHP
B6JKG8, HEM3_HELP2B5ZA04, HEM3_HELPGQ1CUR5, HEM3_HELPH
Q9ZMJ7, HEM3_HELPJB2US69, HEM3_HELPSP56140, HEM3_HELPY
B0UWZ8, HEM3_HISS2P08397, HEM3_HUMANQ31JG5, HEM3_HYDCU
A2BL26, HEM3_HYPBUQ0BX92, HEM3_HYPNAQ5QUS3, HEM3_IDILO
A8AAB4, HEM3_IGNH4B5XYL1, HEM3_KLEP3A6TGI8, HEM3_KLEP7
Q6CT60, HEM3_KLULCQ1INI6, HEM3_KORVEC4Z552, HEM3_LACE2
C1D679, HEM3_LARHHQ1MPL2, HEM3_LAWIPQ5X1F2, HEM3_LEGPA
Q5ZRY6, HEM3_LEGPHQ5WT65, HEM3_LEGPLQ6AHF1, HEM3_LEIXX
A5VM67, HEM3_LIMRDB2G9H4, HEM3_LIMRJQ92BF8, HEM3_LISIN
C1KVK3, HEM3_LISMCQ71ZB2, HEM3_LISMFB8DHJ2, HEM3_LISMH
Q8Y6X5, HEM3_LISMOA0AJ05, HEM3_LISW6B1HVD6, HEM3_LYSSC
B9E743, HEM3_MACCJQ65VX7, HEM3_MANSMA1TXW9, HEM3_MARN8
C6BYB7, HEM3_MARSDQ8TT56, HEM3_METACQ0W5T2, HEM3_METAR
Q46CH0, HEM3_METBFQ12WM8, HEM3_METBUQ602K3, HEM3_METCA
Q1GXA1, HEM3_METFKQ57989, HEM3_METJAQ8TXC8, HEM3_METKA
Q8PW57, HEM3_METMAA4YD92, HEM3_METS5O26960, HEM3_METTH
C5CB88, HEM3_MICLCQ2RJ26, HEM3_MOOTAP22907, HEM3_MOUSE
A0QLI0, HEM3_MYCA1B1MHI5, HEM3_MYCA9P64337, HEM3_MYCBO
A1KFY6, HEM3_MYCBPC1AKJ0, HEM3_MYCBTA4T340, HEM3_MYCGI
Q49808, HEM3_MYCLEB2HRJ2, HEM3_MYCMMQ73SS0, HEM3_MYCPA
A5TZP0, HEM3_MYCTAP9WMP2, HEM3_MYCTOP9WMP3, HEM3_MYCTU
A0PW29, HEM3_MYCUAA1T3D3, HEM3_MYCVPQ1D8Z8, HEM3_MYXXD
B2A1G7, HEM3_NATTJQ9JVS4, HEM3_NEIMAQ9K0P6, HEM3_NEIMB
Q6MW51, HEM3_NEUCRQ82WS2, HEM3_NITEUQ1QQP5, HEM3_NITHX
A9A1A1, HEM3_NITMSA6Q3S1, HEM3_NITSBQ3SVF3, HEM3_NITWN
Q5YP70, HEM3_NOCFAQ8YVU6, HEM3_NOSS1Q8CXC0, HEM3_OCEIH
Q30Y29, HEM3_OLEA2A5CDH4, HEM3_ORITBB3CUP9, HEM3_ORITI
Q6H6D2, HEM3_ORYSJO69110, HEM3_PAEMAA1B853, HEM3_PARDP
Q7U5C2, HEM3_PARMWB2JEN9, HEM3_PARP8B2T1D1, HEM3_PARPJ
Q9CK24, HEM3_PASMUQ43082, HEM3_PEAQ6CZG3, HEM3_PECAS
C6DHC2, HEM3_PECCPB4SFI2, HEM3_PELPBA1AUE7, HEM3_PELPD
A5D3L5, HEM3_PELTSB4RC21, HEM3_PHEZHQ7MYN1, HEM3_PHOLL
Q6L2G8, HEM3_PICTOB4S922, HEM3_PROA2A3PBM0, HEM3_PROM0
A2C0V4, HEM3_PROM1A8G3L1, HEM3_PROM2A2C7L8, HEM3_PROM3
A9BEB6, HEM3_PROM4A2BVF7, HEM3_PROM5Q31C38, HEM3_PROM9
Q7VD89, HEM3_PROMAB4F1X3, HEM3_PROMHQ59684, HEM3_PROMI
Q7V697, HEM3_PROMMQ7V2I1, HEM3_PROMPA2BPX7, HEM3_PROMS
Q46GS7, HEM3_PROMTQ15Z15, HEM3_PSEA6A6VE37, HEM3_PSEA7
B7V5F0, HEM3_PSEA8Q02EA3, HEM3_PSEABQ60169, HEM3_PSEAE
Q1I316, HEM3_PSEE4Q4K3X3, HEM3_PSEF5C3K424, HEM3_PSEFS
A5VWX3, HEM3_PSEP1Q3K4T0, HEM3_PSEPFB0KH05, HEM3_PSEPG
Q88RE5, HEM3_PSEPKB1J1V2, HEM3_PSEPWQ88B91, HEM3_PSESM
Q500N6, HEM3_PSEU2A1T0R0, HEM3_PSYINQ8ZYW7, HEM3_PYRAE
A4WN24, HEM3_PYRARA3MWV7, HEM3_PYRCJA1RQK2, HEM3_PYRIL
B1YDP0, HEM3_PYRNVQ8XWW3, HEM3_RALSOP19356, HEM3_RAT
Q98EI7, HEM3_RHILOQ92LH7, HEM3_RHIMEQ7UPN0, HEM3_RHOBA
C3PNS9, HEM3_RICAEA8GNR5, HEM3_RICAHA8GXC8, HEM3_RICB8
Q1RJN7, HEM3_RICBRA8EYJ9, HEM3_RICCKQ92HR5, HEM3_RICCN
Q4ULA5, HEM3_RICFEA8F1Y8, HEM3_RICM5Q9ZD77, HEM3_RICPR
C4K265, HEM3_RICPUB0BY00, HEM3_RICROA8GSI6, HEM3_RICRS
Q68WR3, HEM3_RICTYA7NKU8, HEM3_ROSCSA5UU37, HEM3_ROSS1
Q1AUK3, HEM3_RUBXDB8I0Q8, HEM3_RUMCHQ59293, HEM3_RUMJO
A1AX51, HEM3_RUTMCQ980U4, HEM3_SACS2A9MJ10, HEM3_SALAR
B5FN93, HEM3_SALDCQ5PKL7, HEM3_SALPAC0Q3B3, HEM3_SALPC
B5BIU8, HEM3_SALPKB4TNV3, HEM3_SALSVP0A1Q9, HEM3_SALTI
P0A1Q8, HEM3_SALTYQ09899, HEM3_SCHPOA8G848, HEM3_SERP5
B8E879, HEM3_SHEB2A3D9Q4, HEM3_SHEB5A6WTG0, HEM3_SHEB8
A9L5G0, HEM3_SHEB9Q088L3, HEM3_SHEFNB0TJ54, HEM3_SHEHH
Q8E9H0, HEM3_SHEONA8GZG4, HEM3_SHEPAA4YBG2, HEM3_SHEPC
A0KS60, HEM3_SHESAA8G0W4, HEM3_SHESHQ0HN99, HEM3_SHESM
Q0HQI7, HEM3_SHESRA1RPD7, HEM3_SHESWB1KQC6, HEM3_SHEWM
Q83PH4, HEM3_SHIFLA9FDP9, 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
Q3BXV3, HEM3_XANC5Q4UYZ4, HEM3_XANC8B0RNI9, HEM3_XANCB
Q8P536, HEM3_XANCPQ2NYU4, 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

View entry in original ENZYME format
View entry in raw text format (no links)
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 2.5.1.-
All ENZYME / UniProtKB/Swiss-Prot entries corresponding to 2.5.-.-
All ENZYME / UniProtKB/Swiss-Prot entries corresponding to 2.-.-.-