Porphobilinogen

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Porphobilinogen

IUPAC name 3-[5-(Aminomethyl)-4-(carboxymethyl)-1H-pyrrol-3-yl]propanoic acid
Identifiers
CAS number	487-90-1
PubChem	1021
	EC number	207-666-3
DrugBank	DB02272
MeSH	Porphobilinogen
Jmol-3D images	Image 1
SMILES C1=C(C(=C(N1)CN)CC(=O)O)CCC(=O)O
Properties
Molecular formula	C₁₀H₁₄N₂O₄
Molar mass	226.229
Y (verify) (what is: Y/N?) Except where noted otherwise, data are given for materials in their standard state (at 25 °C (77 °F), 100 kPa)
Infobox references

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

It is generated by aminolevulinate (ALA) and 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. Cite uses deprecated parameters (help)

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

M: MET

mt, k, c/g/r/p/y/i, f/h/s/l/o/e, a/u, n, m

k, cgrp/y/i, f/h/s/l/o/e, au, n, m, epon

m (A16/C10), i (k, c/g/r/p/y/i, f/h/s/o/e, a/u, n, m)

M: MYL

cell/phys (coag, heme, immu, gran), csfs

rbmg/mogr/tumr/hist, sysi/epon, btst

drug (B1/2/3+5+6), btst, trns

Biochemical families: carbohydrates
- alcohols
- glycoproteins
- glycosides
lipids
- eicosanoids
- fatty acids / intermediates
- phospholipids
- sphingolipids
- steroids
nucleic acids
- constituents / intermediates
proteins
- Amino acids / intermediates
tetrapyrroles / intermediates

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