2.5.1.61: hydroxymethylbilane synthase
This is an abbreviated version!
For detailed information about hydroxymethylbilane synthase, go to the full flat file.
Word Map on EC 2.5.1.61
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2.5.1.61
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porphyria
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intermittent
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heme
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aip
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erythrocyte
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delta-aminolevulinic
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5-aminolevulinic
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gapdh
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erythroid
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genorm
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urinary
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dehydratase
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protoporphyrin
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ferrochelatase
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normfinder
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housekeeping
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tetrapyrrole
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bestkeeper
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coproporphyrinogen
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neurovisceral
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ywhaz
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protoporphyrinogen
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pyrrole
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variegate
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photodynamic
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erythroid-specific
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half-normal
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porphyrinogenic
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coproporphyria
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hematin
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proto
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tarda
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pharmacology
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beta-2-microglobulin
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hyponatremia
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diagnostics
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gata-1
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medicine
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rpl13a
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lightcycler
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cutanea
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beta-globin
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non-erythroid
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reffinder
- 2.5.1.61
- porphyria
-
intermittent
- heme
- aip
- erythrocyte
-
delta-aminolevulinic
-
5-aminolevulinic
- gapdh
-
erythroid
-
genorm
- urinary
- dehydratase
- protoporphyrin
-
ferrochelatase
-
normfinder
-
housekeeping
- tetrapyrrole
-
bestkeeper
- coproporphyrinogen
-
neurovisceral
-
ywhaz
- protoporphyrinogen
- pyrrole
- variegate
-
photodynamic
-
erythroid-specific
-
half-normal
-
porphyrinogenic
- coproporphyria
- hematin
-
proto
- tarda
- pharmacology
-
beta-2-microglobulin
- hyponatremia
- diagnostics
-
gata-1
- medicine
-
rpl13a
-
lightcycler
-
cutanea
- beta-globin
-
non-erythroid
-
reffinder
Reaction
4 porphobilinogen
+
Synonyms
(HMB)-synthase, AN0121.3, EC 4.3.1.8, HemC, hepatic porphobilinogen deaminase, HMB synthase, HMB-S, HMBS, HMBS1a-synthase, HMBS1b-synthase, HMBS2a-synthase, HMBS2b-synthase, human non-erythropoietic PBGD isoform, human PBGD, human porphobilinogen deaminase, hydroxymethylbilane synthase, More, PBG deaminase, PBG-D, PBG-deaminase, PBGD, PGB-D, porphobilinogen ammonia-lyase (polymerizing), porphobilinogen deaminase, pre-uroporphyrinogen synthase, preuroporphyrinogen synthase, preuroporphyrinogen synthetase, synthase, uroporphyrinogen I, uPBGD, UPGI-S, URO-S, urogenI synthase, uroporphyrinogen I synthase, uroporphyrinogen I synthetase, uroporphyrinogen synthase, uroporphyrinogen synthetase
ECTree
2.5.1.61 hydroxymethylbilane synthaseAdvanced search results
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results (Natural Substrates Products
Natural Substrates Products on EC 2.5.1.61 - hydroxymethylbilane synthase
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NATURAL SUBSTRATE 
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
4 porphobilinogen + H2O
1-hydroxymethylbilane + 4 NH3
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third reaction step in heme biosynthesis pathway
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?
4 porphobilinogen + H2O
hydroxymethylbilane + 4 NH3
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?
4 porphobilinogen + H2O
hydroxymethylbilane + 4 NH3
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enzyme defects are involved in acute intermittent porphyria, AIP
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-
?
4 porphobilinogen + H2O
hydroxymethylbilane + 4 NH3
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enzyme defects are involved in acute intermittent porphyria, AIP, a neuropathic disease
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-
?
4 porphobilinogen + H2O
hydroxymethylbilane + 4 NH3
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the enzyme is important in heme biosynthesis, a nonsense mutation in the porphobilinogen deaminase gene causes acute intermittent porphyria, AIP, overview, eight enzymes are involved in the heme synthesis and defects in seven of them cause porphyria, overview
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?
4 porphobilinogen + H2O
hydroxymethylbilane + 4 NH3
the enzyme is important in heme biosynthesis, a nonsense mutation in the porphobilinogen deaminase gene causes chester porphyria, existence of dual porphyrias with two enzymes of heme biosynthesis being deficient simultaneously, overview
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?
4 porphobilinogen + H2O
hydroxymethylbilane + 4 NH3
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third step in the heme biosynthetic pathway, enzyme defects are involved in acute intermittent porphyria, AIP, an autosomal dominant disorder characterised by acute, potentially life-threatening neurological attacks that are precipitated by various drugs, reproductive hormones and other factors
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-
?
4 porphobilinogen + H2O
hydroxymethylbilane + 4 NH3
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?
porphobilinogen
?
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?
porphobilinogen
?
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?
additional information
?
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third enzyme in the heme biosynthetic pathway
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-
?
additional information
?
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in presence of a second enzyme, EC 4.2.1.75, uroporphyrinogen-III synthase, often called co-synthase, the product is cyclized to form uroporphyrinogen-III
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?
additional information
?
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enzyme from chloroplasts is a nuclear-encoded protein, contains a transit-peptide part for transport across the organelle membrans
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?
additional information
?
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third enzyme in the heme biosynthetic pathway
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?
additional information
?
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third enzyme in the heme biosynthetic pathway
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?
additional information
?
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third enzyme in the heme biosynthetic pathway
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?
additional information
?
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third enzyme in the heme biosynthetic pathway
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?
additional information
?
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occurrence of multiple forms of the enzyme. These isomers correspond to the enzyme-substrate intermediates: mono-, di-, tri-, and tetrapyrrole
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?
additional information
?
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mutations of the residues R167, R173, R149, and D99 of the enzyme cause acute intermittent porphyria, overview
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?
additional information
?
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recurrent mutations G111R and R173Q occurring at CpG motifs of the enzyme cause acute intermittent porphyria AIP, a disorder of the heme biosynthetic pathway, oerview
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?
additional information
?
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safety, pharmacokinetics and pharmacodynamics of recombinant human porphobilinogen deaminase P 9808, administered to healthy subjects and asymptomatic porphobilinogen deaminase-deficient subjects with high concentrations of porphobilinogen, the substrate of porphobilinogen deaminase for investigation and establishing of an alternative therapy of acute intermittent porphyria, AIP, method, overview
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?
additional information
?
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the enzyme is also active in uroporphyrinogen formation from porphobilinogen, cf. EC 4.2.1.75
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?
additional information
?
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third enzyme in the heme biosynthetic pathway
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?
additional information
?
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third enzyme in the heme biosynthetic pathway
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?
