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January 16, 2025
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| Id | http://purl.bioontology.org/ontology/MESH/D011163
http://purl.bioontology.org/ontology/MESH/D011163
|
|---|---|
| Preferred Name | Hydroxymethylbilane Synthase |
| Definitions |
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
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| Synonyms |
Synthetase, Preuroporphyrinogen
Porphobilinogen Ammonia Lyase
Hydroxymethylbilane Synthetase
Pre uroporphyrinogen Synthetase
Porphobilinogen ammonia-lyase (polymerizing)
Ammonia-Lyase, Porphobilinogen
Uroporphyrinogen I Synthase
Pre-uroporphyrinogen Synthetase
Deaminase, Porphobilinogen
Synthase, Hydroxymethylbilane
Preuroporphyrinogen Synthetase
Porphobilinogen Deaminase
Synthetase, Hydroxymethylbilane
Synthetase, Pre-uroporphyrinogen
Porphobilinogen Ammonia-Lyase
Synthase, Uroporphyrinogen I
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| Type | http://www.w3.org/2002/07/owl#Class |
All Properties
| definition | 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 |
|---|---|
| altLabel |
Synthetase, Preuroporphyrinogen
Porphobilinogen Ammonia Lyase
Hydroxymethylbilane Synthetase
Pre uroporphyrinogen Synthetase
Porphobilinogen ammonia-lyase (polymerizing)
Ammonia-Lyase, Porphobilinogen
Uroporphyrinogen I Synthase
Pre-uroporphyrinogen Synthetase
Deaminase, Porphobilinogen
Synthase, Hydroxymethylbilane
Preuroporphyrinogen Synthetase
Porphobilinogen Deaminase
Synthetase, Hydroxymethylbilane
Synthetase, Pre-uroporphyrinogen
Porphobilinogen Ammonia-Lyase
Synthase, Uroporphyrinogen I
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| prefLabel |
Hydroxymethylbilane Synthase
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| TH |
NLM (2005)
NLM (1975)
UNK (19XX)
NLM (1998)
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| notation |
D011163
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| EC |
deficiency:Porphyria, Acute Intermittent
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| RR |
9036-47-9 (CAS RN)
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| DX |
19910101
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| Machine permutation |
98; see PORPHOBILINOGEN DEAMINASE 1991-97, see AMMONIA-LYASES 1975-90
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| MN |
D08.811.913.225.575
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| FX |
D011164
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| MMR |
20090706
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| Mapped from | |
| AQL |
AD AE AI AN BI BL CF CH CL CS DE EC GE HI IM IP ME PD PH PK PO RE SD ST TO TU UL UR
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| HN |
98(75)
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| subClassOf | |
| Semantic type UMLS property | |
| Inverse of RB |
EC 2.5.1.61
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| DC |
1
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| MDA |
19741119
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| Inverse of RO | |
| type | |
| tui |
T116
T126
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| cui |
C0032706
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| Inverse of AQ |
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| TERMUI |
T032883
T032885
T032882
T032888
T032881
T032886
T567910
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