porphobilinogen


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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 ?
61-7 U/mL Vitamin D <4 ng/mL 25-80 ng/mL ANA IFA Positive (+) granular pattern Anti-SSA 132(positive) 0-15 Porphobilinogen (total 2900 ml urine) 48.
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.
Decreased PBGD activity can reduce the conversion of porphobilinogen (PBG) into hydroxymethylbilane, which in turn attenuates the feedback inhibition and increases the activity of d-aminolevulinic acid synthase (d-ALAS), thus increasing the levels of ALA and PBG and consequently urinary excretion thereof.
3), (4) In our case, diagnosis was made after the recognition of dark urine and higher than normal urine porphobilinogen, corpoporphyrin and total porphyrin levels.
Motor neuropathy in porphobilinogen deaminase-deficient mice imitates the peripheral neuropathy of human acute porphyria.
It is a metabolic error that affects more women than men and is inherited in autosomal dominant manner, causing a partial deficit in the porphobilinogen deaminase enzyme.
Cis- and transacting elements involved in the regulation of the erythroid promoter of the human porphobilinogen deaminase gene.
Succinate and glycine condense to form aminolevulinate, and two molecules of this intermediate condense to form porphobilinogen.
4]) normal normal Liver function tests normal normal Urine porphobilinogen negative negative Creatine phosphokinase normal normal Table 2 Complementary laboratory tests Complementary laboratory tests Patient 1 Patient 2 HIV negative negative Hepatitis B antibody negative negative VDRL negative negative Campylobacter jejuni (serology) negative negative Viral titres (Cytomegalovirus) negative negative Antinuclear factors negative negative Table 3 Neurological recovery Recovery pattern Patient 1 Patient 2 (weeks) (weeks) Pupillary reaction 2 8 Eye movements 4 10 Swallowing 6 14 Response to pain 5 10 Weaned from ventilatory support 12 16
Background: Acute Intermittent Porphyria (AIP) is a rare autosomal dominant metabolic disorder resulting from partial deficiency of porphobilinogen deaminase, the third enzyme of the haem synthetic pathway.
If the excess is of the early precursor molecules (delta aminolevulenic acid [ALA], or porphobilinogen [PBG], or both), the manifestations are neurovisceral.