Porphobilinogen

Porphobilinogen
Names

IUPAC name 3-[5-(Aminomethyl)-4-(carboxymethyl)-1H-pyrrol-3-yl]propanoic acid
Identifiers
CAS Number	487-90-1^Y
ChemSpider	995^N
DrugBank	DB02272^Y
EC Number	207-666-3
Jmol 3D model	Interactive image
MeSH	Porphobilinogen
PubChem	1021
UNII	74KHC72QXK^N
InChI InChI=1S/C10H14N2O4/c11-4-8-7(3-10(15)16)6(5-12-8)1-2-9(13)14/h5,12H,1-4,11H2,(H,13,14)(H,15,16)^N Key: QSHWIQZFGQKFMA-UHFFFAOYSA-N^N InChI=1/C10H14N2O4/c11-4-8-7(3-10(15)16)6(5-12-8)1-2-9(13)14/h5,12H,1-4,11H2,(H,13,14)(H,15,16) Key: QSHWIQZFGQKFMA-UHFFFAOYAF
SMILES C1=C(C(=C(N1)CN)CC(=O)O)CCC(=O)O
Properties
Chemical formula	C₁₀H₁₄N₂O₄
Molar mass	226.229
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).
N verify (what is ^YN ?)
Infobox references

Porphobilinogen (PBG) is a pyrrole involved in porphyrin metabolism.

It is generated from aminolevulinate (ALA) by the enzyme ALA dehydratase. PBG is then converted into hydroxymethyl bilane by the enzyme porphobilinogen deaminase, also known as hydroxymethylbilane synthase.

Acute intermittent porphyria causes an increase in urinary porphobilinogen.^[1]

References

↑ Aarsand, AK; Petersen PH; Sandberg S (April 2006). "Estimation and application of biological variation of urinary delta-aminolevulinic acid and porphobilinogen in healthy individuals and in patients with acute intermittent porphyria". Clinical Chemistry 52 (4): 650–656. doi:10.1373/clinchem.2005.060772. PMID 16595824.

Heme metabolic intermediates

Porphyrin biosynthesis

early mitochondrial:	D-Aminolevulinic acid

cytosolic:	Porphobilinogen Hydroxymethylbilane Uroporphyrinogen III Coproporphyrinogen III

late mitochondrial:	Protoporphyrinogen IX Protoporphyrin IX

Heme degradation
and excretion

Breakdown of heme

spleen:	Heme → Biliverdin → Bilirubin

blood:	Bilirubin+Albumin

liver:	Bilirubin diglucuronide

Intestine, excretion in feces

Kidney, excretion in urine

Urobilin

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