MeSH Browser

MeSH Heading: Hydroxymethylbilane Synthase
Tree Number(s): D08.811.913.225.575
Unique ID: D011163
RDF Unique Identifier: http://id.nlm.nih.gov/mesh/D011163
Scope Note: An enzyme that catalyzes the tetrapolymerization of the monopyrrole PORPHOBILINOGEN into the hydroxymethylbilane preuroporphyrinogen (UROPORPHYRINOGENS) in several discrete steps. It is the third enzyme in the 8-enzyme biosynthetic pathway of HEME. In humans, deficiency in this enzyme encoded by HMBS (or PBGD) gene results in a form of neurological porphyria (PORPHYRIA, ACUTE INTERMITTENT). This enzyme was formerly listed as EC 4.3.1.8
Entry Term(s): Hydroxymethylbilane Synthetase; Porphobilinogen Ammonia-Lyase; Porphobilinogen Deaminase; Pre-uroporphyrinogen Synthetase; Preuroporphyrinogen Synthetase; Uroporphyrinogen I Synthase
Related Numbers: 9036-47-9
CAS Type 1 Name: Porphobilinogen ammonia-lyase (polymerizing)
Previous Indexing: Ammonia-Lyases (1973-1974); Porphobilinogen (1973-1974)
See Also: Porphyrias
Public MeSH Note: 98; see PORPHOBILINOGEN DEAMINASE 1991-97, see AMMONIA-LYASES 1975-90
History Note: 98(75)
Entry Combination: deficiency:Porphyria, Acute Intermittent
Date Established: 1991/01/01
Date of Entry: 1974/11/19
Revision Date: 2009/07/06

Entry Combination: deficiency:Porphyria, Acute Intermittent
Allowable Qualifiers: administration & dosage (AD)

adverse effects (AE)

analysis (AN)

antagonists & inhibitors (AI)

biosynthesis (BI)

blood (BL)

cerebrospinal fluid (CF)

chemical synthesis (CS)

chemistry (CH)

classification (CL)

drug effects (DE)

economics (EC)

genetics (GE)

history (HI)

immunology (IM)

isolation & purification (IP)

metabolism (ME)

pharmacokinetics (PK)

pharmacology (PD)

physiology (PH)

poisoning (PO)

radiation effects (RE)

standards (ST)

supply & distribution (SD)

therapeutic use (TU)

toxicity (TO)

ultrastructure (UL)

urine (UR)

Concept UI: M0017332
Related Numbers: 9036-47-9
Scope Note: An enzyme that catalyzes the tetrapolymerization of the monopyrrole PORPHOBILINOGEN into the hydroxymethylbilane preuroporphyrinogen (UROPORPHYRINOGENS) in several discrete steps. It is the third enzyme in the 8-enzyme biosynthetic pathway of HEME. In humans, deficiency in this enzyme encoded by HMBS (or PBGD) gene results in a form of neurological porphyria (PORPHYRIA, ACUTE INTERMITTENT). This enzyme was formerly listed as EC 4.3.1.8
Terms: Hydroxymethylbilane Synthase Preferred Term
Term UI T032881
Date01/01/1999
LexicalTag NON
ThesaurusID NLM (1975); Porphobilinogen Ammonia-Lyase
Term UI T032882
Date11/11/1974
LexicalTag NON
ThesaurusID UNK (19XX); Porphobilinogen Deaminase
Term UI T032883
Date05/23/1997
LexicalTag NON
ThesaurusID NLM (1998); Hydroxymethylbilane Synthetase
Term UI T032885
Date03/05/1984
LexicalTag NON
ThesaurusID UNK (19XX); Preuroporphyrinogen Synthetase
Term UI T032886
Date08/04/1980
LexicalTag NON
ThesaurusID UNK (19XX); Uroporphyrinogen I Synthase
Term UI T032888
Date04/16/1984
LexicalTag NON
ThesaurusID UNK (19XX); Pre-uroporphyrinogen Synthetase
Term UI T567910
Date01/08/2004
LexicalTag NON
ThesaurusID NLM (2005)

Hydroxymethylbilane Synthase MeSH Descriptor Data 2025