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Porphobilinogen deaminase (hydroxymethylbilane synthase, or
uroporphyrinogen I synthase) is an
enzyme (EC 2.5.1.61) that in
humans is
encoded by the HMBS...
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Porphobilinogen (PBG) is an
organic compound that
occurs in
living organisms as an
intermediate in the
biosynthesis of porphyrins,
which include critical...
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Aminolevulinic acid
dehydratase (
porphobilinogen synthase, or ALA dehydratase, or
aminolevulinate dehydratase) is an
enzyme (EC 4.2.1.24) that in humans...
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affecting the
production of heme
resulting from a
deficiency of the
enzyme porphobilinogen deaminase. It is the most
common of the
acute porphyrias. The clinical...
- pathway. Two
molecules of dALA are then
combined by
porphobilinogen synthase to give
porphobilinogen (PBG),
which contains a
pyrrole ring. Four PBGs are...
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derivatives are also
called pyrroles, e.g., N-methylpyrrole, C4H4NCH3.
Porphobilinogen, a
trisubstituted pyrrole, is the
biosynthetic precursor to many natural...
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undergo a
condensation reaction catalyzed by
Porphobilinogen (PBG)
Synthase to
yield a
molecule of
Porphobilinogen (PBG) (not shown). Four
molecules of PBG...
- enzymes, such as zinc
cations in the ALAD enzyme,
which is also
known as
porphobilinogen synthase, and is
important in the
synthesis of heme, a key component...
- a
number of enzymes,
including δ-aminolevulinic acid
dehydratase (
porphobilinogen synthase) in the haem
biosynthetic pathway and pyrimidine-5′-nucleotidase...
-
analysis of blood, urine, and stool. In general,
urine estimation of
porphobilinogen (PBG) is the
first step if
acute porphyria is suspected. As a result...
