porphobilinogen

(redirected from Porphobilinogen deaminase)
Also found in: Encyclopedia, Wikipedia.

porphobilinogen

 [por″fo-bĭ-lin´o-jen]
an intermediary product in the biosynthesis of heme.

por·pho·bi·lin·o·gen (PBG),

(pōr'fō-bī-lin'ō-jen),
A porphyrin precursor of porphyrinogens, porphyrins, and heme; found in the urine in large quantities in cases of acute or congenital porphyria.

porphobilinogen

/por·pho·bi·lin·o·gen/ (por″fo-bĭ-lin´ah-jin) an intermediary product in the biosynthesis of heme; it is produced in excess and excreted in the urine in acute intermittent porphyria.

porphobilinogen

[pôr′fōbilin′əjən]
a chromogen substance that is an intermediate in the biosynthesis of heme and porphyrins. It appears in the urine of people with porphyria, representing an error of metabolism. See also heme, porphyria.

por·pho·bi·lin·o·gen

(PBG) (pōr'fō-bī-lin'ŏ-jen)
A porphyrin precursor of porphyrinogens, porphyrins, and heme; found in the urine in large quantities in cases of acute or congenital porphyria.

porphobilinogen

an intermediary product in the biosynthesis of heme. Not detectable in normal animals.

porphobilinogen deaminase
an enzyme involved in the condensation of porphobilinogen. Now called uroporphyrinogen I synthetase.
References in periodicals archive ?
Some research results on rice and Arabidopsis thaliana found that the formation of chlorophyll-deficient mutant was the block of chlorophyll biosynthesis regulated by enzymes of D-aminolevulinate dehydratase (ALAD), porphobilinogen deaminase (PBGD) and magnesium chelatase (Mg-chelatase) (Rissler et al.
Acute intermittent porphyria in Sweden: Molecular, functional and clinical consequences of some new mutations found in the porphobilinogen deaminase gene.
1 Acute intermittent porphyria (AIP), an autosomal dominant disorder, is a common type of neurologic porphyria in which mutation of the porphobilinogen deaminase (PBGD) gene plays an important role.
For confirmatory diagnosis, porphobilinogen deaminase (PBGD) test was done which was found to be positive.
Acute intermittent porphyria (AIP) is the most common of the acute hepatic porphyrias, and results from partial deficiency of porphobilinogen deaminase, the third enzyme of the haem synthetic pathway [Badminton and Elder, 2002].
Some research results on rice and Arabidopsis thaliana found that the formation of chlorophyll-deficient mutant was the block of chlorophyll biosynthesis regulated by enzymes of d- aminolevulinate dehydratase (ALAD), porphobilinogen deaminase (PBGD) and magnesium chelatase (Mg- chelatase) (Rissler et al.
In distribution E, 13 of the 15 reported results were belowthe lower reference limits for porphobilinogen deaminase (PBGD), and 2 of 4 for uroporphyrinogen decarboxylase (UROD).
In preliminary experiments, amplification factors were determined for specific target sequences Mus musculus glyceraldehyde-3-phosphate dehydrogenase gene 3' untranslated region (Gapd 3'-UTR), Gapd, and Mus musculus porphobilinogen deaminase (Pbgd).
Acute intermittent porphyria (AIP) (4) is an autosomal dominant disorder caused by a metabolic error in heme biosynthesis in which the third enzyme, porphobilinogen deaminase (PBGD; EC 2.
Some consistency in enzyme nomenclature is called for; uroporphyrinogen I synthase and porphobilinogen deaminase should be confined to the dust bin of history--hydroxymethyl bilane synthase is the correct and internationally preferred term.
Acute intermittent porphyria (AIP) [1] is a metabolic disease that results from the partial deficiency of porphobilinogen deaminase (PBGD; also known as hydroxymethylbilane synthase; EC 4.
The porphobilinogen deaminase (PBGD) housekeeping gene was used as endogenous control to compensate for different degrees of inhibition during reverse transcription and PCR (6).