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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
4 porphobilinogen + H(2)O <=> hydroxymethylbilane + 4 NH(3)
Cofactor(s)
Dipyrromethane.
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
PROSITEPDOC00461
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
UniProtKB/Swiss-Prot
Q82E76, HEM31_STRAW;  Q9WX16, HEM31_STRCO;  Q82P95, HEM32_STRAW;  
Q9KY00, HEM32_STRCO;  Q6FFA9, HEM3_ACIAD;  A0LRF1, HEM3_ACIC1;  
B7JBH8, HEM3_ACIF2;  B5ERN6, HEM3_ACIF5;  A6VL87, HEM3_ACTSZ;  
Q9Y9J0, HEM3_AERPE;  Q8UC46, HEM3_AGRFC;  Q0VM28, HEM3_ALCBS;  
B6EPK1, HEM3_ALISL;  Q0A577, HEM3_ALKEH;  A6TJD5, HEM3_ALKMQ;  
A8MGE4, HEM3_ALKOO;  O66621, HEM3_AQUAE;  Q43316, HEM3_ARATH;  
A8ERI4, HEM3_ARCB4;  O29026, HEM3_ARCFU;  Q75DY0, HEM3_ASHGO;  
C3P9E9, HEM3_BACAA;  C3L6Z5, HEM3_BACAC;  A0RJ81, HEM3_BACAH;  
Q81LC7, HEM3_BACAN;  B7JQ57, HEM3_BACC0;  Q72ZW2, HEM3_BACC1;  
B7IIX5, HEM3_BACC2;  C1ETR0, HEM3_BACC3;  B7HE96, HEM3_BACC4;  
B7HQM4, HEM3_BACC7;  A7GTE5, HEM3_BACCN;  B9IZ39, HEM3_BACCQ;  
Q817R0, HEM3_BACCR;  Q633Y0, HEM3_BACCZ;  Q9K8G0, HEM3_BACHD;  
Q6HD62, HEM3_BACHK;  Q65GK1, HEM3_BACLD;  A8FFV2, HEM3_BACP2;  
Q5WEP5, HEM3_BACSK;  P16616, HEM3_BACSU;  A7Z7A5, HEM3_BACVZ;  
A9VIT8, HEM3_BACWK;  Q1LU25, HEM3_BAUCH;  Q7VRM4, HEM3_BLOFL;  
Q491Z6, HEM3_BLOPB;  Q7WKM1, HEM3_BORBR;  Q7W785, HEM3_BORPA;  
A9IKF3, HEM3_BORPD;  Q7VVU9, HEM3_BORPE;  Q2KIN5, HEM3_BOVIN;  
C0ZAH1, HEM3_BREBN;  B2S842, HEM3_BRUA1;  Q2YLM5, HEM3_BRUA2;  
Q57B08, HEM3_BRUAB;  A9M8M2, HEM3_BRUC2;  C0RFD1, HEM3_BRUMB;  
Q8YJB0, HEM3_BRUME;  A5VSL5, HEM3_BRUO2;  B0CID9, HEM3_BRUSI;  
Q8FYI6, HEM3_BRUSU;  B8D8F3, HEM3_BUCA5;  P57651, HEM3_BUCAI;  
B8D8B0, HEM3_BUCAT;  B1JWR0, HEM3_BURCC;  B4E8C4, HEM3_BURCJ;  
Q0BCU9, HEM3_BURCM;  A3MLK5, HEM3_BURM7;  A2SAH5, HEM3_BURM9;  
Q62LC0, HEM3_BURMA;  A1V5U0, HEM3_BURMS;  A3NSN6, HEM3_BURP0;  
Q3JUW3, HEM3_BURP1;  A3N700, HEM3_BURP6;  Q63W73, HEM3_BURPS;  
Q2T071, HEM3_BURTA;  A4JGV4, HEM3_BURVG;  A8MDU3, HEM3_CALMQ;  
A4XK06, HEM3_CALS8;  A7ZD37, HEM3_CAMC1;  A7GYU4, HEM3_CAMC5;  
A0RNT3, HEM3_CAMFF;  A7I1S0, HEM3_CAMHC;  A8FKW8, HEM3_CAMJ8;  
A7H4J7, HEM3_CAMJD;  Q9PHW9, HEM3_CAMJE;  A1VYQ4, HEM3_CAMJJ;  
Q5HVM1, HEM3_CAMJR;  B9KGA9, HEM3_CAMLR;  O94048, HEM3_CANAL;  
Q6FNR4, HEM3_CANGA;  Q3ACT3, HEM3_CARHZ;  B8GXG1, HEM3_CAUCN;  
Q9ABZ8, HEM3_CAUCR;  B0T287, HEM3_CAUSK;  Q3AQF5, HEM3_CHLCH;  
B3EIN1, HEM3_CHLL2;  Q3B2Z5, HEM3_CHLL7;  Q9PK95, HEM3_CHLMU;  
P28464, HEM3_CHLP8;  B3EPK0, HEM3_CHLPB;  A1BHD6, HEM3_CHLPD;  
A4SFJ8, HEM3_CHLPM;  Q9Z9C9, HEM3_CHLPN;  B3QWI1, HEM3_CHLT3;  
Q8KCJ4, HEM3_CHLTE;  O84301, HEM3_CHLTR;  Q7P207, HEM3_CHRVO;  
Q97MU4, HEM3_CLOAB;  A7FSG8, HEM3_CLOB1;  C3L2Z3, HEM3_CLOB6;  
A6LSY5, HEM3_CLOB8;  B2UXC4, HEM3_CLOBA;  B2TPD7, HEM3_CLOBB;  
A5I0B2, HEM3_CLOBH;  C1FVB9, HEM3_CLOBJ;  B1IHD1, HEM3_CLOBK;  
A7GBW3, HEM3_CLOBL;  B1KY15, HEM3_CLOBM;  B8I0Q8, HEM3_CLOCE;  
Q59293, HEM3_CLOJO;  B9DZL6, HEM3_CLOK1;  A0Q2B1, HEM3_CLONN;  
Q0TQG4, HEM3_CLOP1;  Q8XKG4, HEM3_CLOPE;  Q0ST16, HEM3_CLOPS;  
A3DIF0, HEM3_CLOTH;  Q48AW8, HEM3_COLP3;  C3PKG2, HEM3_CORA7;  
Q6NJK0, HEM3_CORDI;  Q8FSF7, HEM3_COREF;  A4QB59, HEM3_CORGB;  
Q8NT90, HEM3_CORGL;  B6J6K0, HEM3_COXB1;  B6J3T6, HEM3_COXB2;  
A9KDC4, HEM3_COXBN;  A9N910, HEM3_COXBR;  Q83A37, HEM3_COXBU;  
A7MQJ1, HEM3_CROS8;  Q11XT5, HEM3_CYTH3;  Q6BM23, HEM3_DEBHA;  
Q479S8, HEM3_DECAR;  C1CYD9, HEM3_DEIDV;  Q1J288, HEM3_DEIGD;  
Q9RRY0, HEM3_DEIRA;  C6BYB7, HEM3_DESAD;  Q30Y29, HEM3_DESAG;  
B1I4L8, HEM3_DESAP;  B8G2M2, HEM3_DESHD;  Q24VC8, HEM3_DESHY;  
A8ZWG5, HEM3_DESOH;  A4J6H7, HEM3_DESRM;  Q72AV0, HEM3_DESVH;  
B8DKW2, HEM3_DESVM;  A1VCX6, HEM3_DESVV;  P40129, HEM3_DICCH;  
Q54P93, HEM3_DICDI;  A5EW83, HEM3_DICNV;  B7MH66, HEM3_ECO45;  
B7L959, HEM3_ECO55;  Q8XAP3, HEM3_ECO57;  B7NTE0, HEM3_ECO7I;  
B7N295, HEM3_ECO81;  B7M603, HEM3_ECO8A;  C4ZZ69, HEM3_ECOBW;  
B1XAH2, HEM3_ECODH;  Q8FBP1, HEM3_ECOL6;  P06983, HEM3_ECOLI;  
B7NFA7, HEM3_ECOLU;  B6I4E0, HEM3_ECOSE;  Q3YSA8, HEM3_EHRCJ;  
Q2GGC7, HEM3_EHRCR;  A4WG12, HEM3_ENT38;  B2VG55, HEM3_ERWT9;  
B7LU53, HEM3_ESCF3;  C4Z552, HEM3_EUBE2;  P13446, HEM3_EUGGR;  
B1YJV1, HEM3_EXIS2;  C4L4J6, HEM3_EXISA;  B0TW04, HEM3_FRAP2;  
Q14JI3, HEM3_FRAT1;  A7N9H6, HEM3_FRATF;  Q2A5R3, HEM3_FRATH;  
B2SE34, HEM3_FRATM;  A0Q481, HEM3_FRATN;  Q0BP15, HEM3_FRATO;  
Q5NI31, HEM3_FRATT;  A4IZY4, HEM3_FRATW;  Q8RFP5, HEM3_FUSNN;  
B5EBG8, HEM3_GEOBB;  B9M416, HEM3_GEODF;  Q5KWK6, HEM3_GEOKA;  
B3E2H7, HEM3_GEOLS;  Q39QM7, HEM3_GEOMG;  Q747I1, HEM3_GEOSL;  
C6DYY8, HEM3_GEOSM;  C5D5K7, HEM3_GEOSW;  A4IRG5, HEM3_GEOTN;  
A5GCW2, HEM3_GEOUR;  Q7NGF7, HEM3_GLOVI;  Q0I178, HEM3_HAES1;  
Q2SQ70, HEM3_HAHCH;  A1WVT9, HEM3_HALHL;  Q9HMY5, HEM3_HALSA;  
C4K4E3, HEM3_HAMD5;  Q17W61, HEM3_HELAH;  Q7VFE9, HEM3_HELHP;  
B6JKG8, HEM3_HELP2;  B5ZA04, HEM3_HELPG;  Q1CUR5, HEM3_HELPH;  
Q9ZMJ7, HEM3_HELPJ;  B2US69, HEM3_HELPS;  P56140, HEM3_HELPY;  
B0UWZ8, HEM3_HISS2;  P08397, HEM3_HUMAN;  A2BL26, HEM3_HYPBU;  
Q0BX92, HEM3_HYPNA;  Q5QUS3, HEM3_IDILO;  A8AAB4, HEM3_IGNH4;  
B5XYL1, HEM3_KLEP3;  A6TGI8, HEM3_KLEP7;  Q6CT60, HEM3_KLULA;  
Q1INI6, HEM3_KORVE;  A5VM67, HEM3_LACRD;  B2G9H4, HEM3_LACRJ;  
C1D679, HEM3_LARHH;  Q1MPL2, HEM3_LAWIP;  Q5X1F2, HEM3_LEGPA;  
Q5ZRY6, HEM3_LEGPH;  Q5WT65, HEM3_LEGPL;  Q6AHF1, HEM3_LEIXX;  
Q92BF8, HEM3_LISIN;  C1KVK3, HEM3_LISMC;  Q71ZB2, HEM3_LISMF;  
B8DHJ2, HEM3_LISMH;  Q8Y6X5, HEM3_LISMO;  A0AJ05, HEM3_LISW6;  
B1HVD6, HEM3_LYSSC;  B9E743, HEM3_MACCJ;  Q65VX7, HEM3_MANSM;  
A1TXW9, HEM3_MARHV;  Q8TT56, HEM3_METAC;  Q0W5T2, HEM3_METAR;  
Q46CH0, HEM3_METBF;  Q12WM8, HEM3_METBU;  Q602K3, HEM3_METCA;  
Q1GXA1, HEM3_METFK;  Q57989, HEM3_METJA;  Q8TXC8, HEM3_METKA;  
Q8PW57, HEM3_METMA;  A4YD92, HEM3_METS5;  O26960, HEM3_METTH;  
C5CB88, HEM3_MICLC;  Q2RJ26, HEM3_MOOTA;  P22907, HEM3_MOUSE;  
A0QLI0, HEM3_MYCA1;  B1MHI5, HEM3_MYCA9;  P64337, HEM3_MYCBO;  
A1KFY6, HEM3_MYCBP;  C1AKJ0, HEM3_MYCBT;  A4T340, HEM3_MYCGI;  
Q49808, HEM3_MYCLE;  B2HRJ2, HEM3_MYCMM;  Q73SS0, HEM3_MYCPA;  
A5TZP0, HEM3_MYCTA;  P9WMP2, HEM3_MYCTO;  P9WMP3, HEM3_MYCTU;  
A0PW29, HEM3_MYCUA;  A1T3D3, HEM3_MYCVP;  Q1D8Z8, HEM3_MYXXD;  
B2A1G7, HEM3_NATTJ;  Q9JVS4, HEM3_NEIMA;  Q9K0P6, HEM3_NEIMB;  
Q6MW51, HEM3_NEUCR;  Q82WS2, HEM3_NITEU;  Q1QQP5, HEM3_NITHX;  
A9A1A1, HEM3_NITMS;  A6Q3S1, HEM3_NITSB;  Q3SVF3, HEM3_NITWN;  
Q5YP70, HEM3_NOCFA;  Q8YVU6, HEM3_NOSS1;  Q8CXC0, HEM3_OCEIH;  
A6WXJ2, HEM3_OCHA4;  A5CDH4, HEM3_ORITB;  B3CUP9, HEM3_ORITI;  
Q6H6D2, HEM3_ORYSJ;  O69110, HEM3_PAEMA;  A1B853, HEM3_PARDP;  
B2JEN9, HEM3_PARP8;  B2T1D1, HEM3_PARPJ;  Q9CK24, HEM3_PASMU;  
Q43082, HEM3_PEA;  Q6CZG3, HEM3_PECAS;  C6DHC2, HEM3_PECCP;  
Q3A009, HEM3_PELCD;  B4SFI2, HEM3_PELPB;  A1AUE7, HEM3_PELPD;  
A5D3L5, HEM3_PELTS;  Q180R9, HEM3_PEPD6;  B4RC21, HEM3_PHEZH;  
Q7MYN1, HEM3_PHOLL;  Q6L2G8, HEM3_PICTO;  B4S922, HEM3_PROA2;  
Q6AB05, HEM3_PROAC;  A3PBM0, HEM3_PROM0;  A2C0V4, HEM3_PROM1;  
A8G3L1, HEM3_PROM2;  A2C7L8, HEM3_PROM3;  A9BEB6, HEM3_PROM4;  
A2BVF7, HEM3_PROM5;  Q31C38, HEM3_PROM9;  Q7VD89, HEM3_PROMA;  
B4F1X3, HEM3_PROMH;  Q59684, HEM3_PROMI;  Q7V697, HEM3_PROMM;  
Q7V2I1, HEM3_PROMP;  A2BPX7, HEM3_PROMS;  Q46GS7, HEM3_PROMT;  
Q15Z15, HEM3_PSEA6;  A6VE37, HEM3_PSEA7;  B7V5F0, HEM3_PSEA8;  
Q02EA3, HEM3_PSEAB;  Q60169, HEM3_PSEAE;  Q1I316, HEM3_PSEE4;  
Q4K3X3, HEM3_PSEF5;  C3K424, HEM3_PSEFS;  A5VWX3, HEM3_PSEP1;  
Q3K4T0, HEM3_PSEPF;  B0KH05, HEM3_PSEPG;  Q88RE5, HEM3_PSEPK;  
B1J1V2, HEM3_PSEPW;  Q88B91, HEM3_PSESM;  Q500N6, HEM3_PSEU2;  
A1T0R0, HEM3_PSYIN;  Q8ZYW7, HEM3_PYRAE;  A4WN24, HEM3_PYRAR;  
A3MWV7, HEM3_PYRCJ;  A1RQK2, HEM3_PYRIL;  B1YDP0, HEM3_PYRNV;  
Q8XWW3, HEM3_RALSO;  P19356, HEM3_RAT;  Q98EI7, HEM3_RHILO;  
Q92LH7, HEM3_RHIME;  Q7UPN0, HEM3_RHOBA;  Q21XQ9, HEM3_RHOFT;  
C3PNS9, HEM3_RICAE;  A8GNR5, HEM3_RICAH;  A8GXC8, HEM3_RICB8;  
Q1RJN7, HEM3_RICBR;  A8EYJ9, HEM3_RICCK;  Q92HR5, HEM3_RICCN;  
Q4ULA5, HEM3_RICFE;  A8F1Y8, HEM3_RICM5;  Q9ZD77, HEM3_RICPR;  
C4K265, HEM3_RICPU;  B0BY00, HEM3_RICRO;  A8GSI6, HEM3_RICRS;  
Q68WR3, HEM3_RICTY;  A7NKU8, HEM3_ROSCS;  A5UU37, HEM3_ROSS1;  
Q1AUK3, HEM3_RUBXD;  A1AX51, HEM3_RUTMC;  A9MJ10, HEM3_SALAR;  
B5FN93, HEM3_SALDC;  Q5PKL7, HEM3_SALPA;  C0Q3B3, HEM3_SALPC;  
B5BIU8, HEM3_SALPK;  B4TNV3, HEM3_SALSV;  P0A1Q9, HEM3_SALTI;  
P0A1Q8, HEM3_SALTY;  Q09899, HEM3_SCHPO;  A8G848, HEM3_SERP5;  
B8E879, HEM3_SHEB2;  A3D9Q4, HEM3_SHEB5;  A6WTG0, HEM3_SHEB8;  
A9L5G0, HEM3_SHEB9;  Q088L3, HEM3_SHEFN;  B0TJ54, HEM3_SHEHH;  
Q8E9H0, HEM3_SHEON;  A8GZG4, HEM3_SHEPA;  A4YBG2, HEM3_SHEPC;  
A0KS60, HEM3_SHESA;  A8G0W4, HEM3_SHESH;  Q0HN99, HEM3_SHESM;  
Q0HQI7, HEM3_SHESR;  A1RPD7, HEM3_SHESW;  B1KQC6, HEM3_SHEWM;  
Q83PH4, HEM3_SHIFL;  A9FDP9, HEM3_SORC5;  Q1GP41, HEM3_SPHAL;  
A7X391, HEM3_STAA1;  A6U2D8, HEM3_STAA2;  Q2FG66, HEM3_STAA3;  
O34090, HEM3_STAA8;  A5ITJ5, HEM3_STAA9;  Q2YTB9, HEM3_STAAB;  
Q5HFA2, HEM3_STAAC;  A6QHK4, HEM3_STAAE;  P64340, HEM3_STAAM;  
P64341, HEM3_STAAN;  Q6GG35, HEM3_STAAR;  Q6G8Q5, HEM3_STAAS;  
A8Z2J1, HEM3_STAAT;  Q8NW74, HEM3_STAAW;  B9DNE1, HEM3_STACT;  
Q5HNN3, HEM3_STAEQ;  Q8CNY8, HEM3_STAES;  Q4L709, HEM3_STAHJ;  
Q49YA2, HEM3_STAS1;  B4SJT8, HEM3_STRM5;  B2FI06, HEM3_STRMK;  
A3CL78, HEM3_STRSV;  Q30S90, HEM3_SULDN;  C3MZR8, HEM3_SULIA;  
C4KIZ9, HEM3_SULIK;  C3MJ21, HEM3_SULIL;  C3MYD9, HEM3_SULIM;  
C3NF70, HEM3_SULIN;  C3N841, HEM3_SULIY;  A6Q7Y4, HEM3_SULNB;  
Q980U4, HEM3_SULSO;  Q976H1, HEM3_SULTO;  Q2LQU2, HEM3_SYNAS;  
Q31PM2, HEM3_SYNE7;  A0LN73, HEM3_SYNFM;  Q7U5C2, HEM3_SYNPX;  
A5GSE0, HEM3_SYNR3;  Q0I8I3, HEM3_SYNS3;  Q3AWQ4, HEM3_SYNS9;  
Q3ALS9, HEM3_SYNSC;  Q0AZ43, HEM3_SYNWW;  P73660, HEM3_SYNY3;  
Q9HKM5, HEM3_THEAC;  Q8DIE4, HEM3_THEEB;  Q47LA7, HEM3_THEFY;  
Q72H57, HEM3_THET2;  Q5SLE8, HEM3_THET8;  Q97B26, HEM3_THEVO;  
Q31JG5, HEM3_THICR;  Q3SFT5, HEM3_THIDA;  B8GSU8, HEM3_THISH;  
A5CW34, HEM3_VESOH;  A7MXU9, HEM3_VIBCB;  Q9KVM1, HEM3_VIBCH;  
Q5E8T5, HEM3_VIBF1;  B5FF97, HEM3_VIBFM;  Q87KI9, HEM3_VIBPA;  
B7VMD9, HEM3_VIBTL;  Q8DD85, HEM3_VIBVU;  Q7MQC7, HEM3_VIBVY;  
Q7M8L2, HEM3_WOLSU;  C0R2M3, HEM3_WOLWR;  Q8PPR3, HEM3_XANAC;  
Q3BXV3, HEM3_XANC5;  Q4UYZ4, HEM3_XANC8;  B0RNI9, HEM3_XANCB;  
Q8P536, HEM3_XANCP;  Q2NYU4, HEM3_XANOM;  B2SMV3, HEM3_XANOP;  
Q5GVL2, HEM3_XANOR;  B2I5K8, HEM3_XYLF2;  Q9PCX7, HEM3_XYLFA;  
B0U2W8, HEM3_XYLFM;  Q87CC9, HEM3_XYLFT;  Q6C097, HEM3_YARLI;  
P28789, HEM3_YEAST;  A1JI86, HEM3_YERE8;  P30527, HEM3_YERIN;  
A7FD67, HEM3_YERP3;  Q1CBN1, HEM3_YERPA;  B2K065, HEM3_YERPB;  
P46355, HEM3_YERPE;  A9R8K0, HEM3_YERPG;  Q1CNI3, HEM3_YERPN;  
A4TRA8, HEM3_YERPP;  Q66G00, HEM3_YERPS;  B1JPE6, HEM3_YERPY;  

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