Porphobilinogen
Porphobilinogen is a pyrrole involved in porphyrin metabolism. — Wikipedia; It consists of a pyrrole ring with acetyl, propionyl, and aminomethyl side chains; It is a key monopyrrolic intermediate in porphyrin, chlorophyll and vitamin B12 biosynthesis. Porphobilinogen is generated by the enzyme ALA dehydratase by combining two moleclies of dALA together, and converted into hydroxymethyl bilane by the enzyme porphobilinogen deaminase. 4 moleclies of porphobilinogen are condensed to form one moleclie of uroporphyrinogen III, which is then converted successively to coproporphyrinogen III, protoporphyrin IX, and heme. Porphobilinogen is produced in excess and excreted in the urine in acute intermittent porphyria and several other porphyrias.
Structure for HMDB00245 (Porphobilinogen)
C10H14N2O4
226.2292
226.095356946
3-[5-(aminomethyl)-4-(carboxymethyl)-1H-pyrrol-3-yl]propanoic acid
porphobilinogen
487-90-1
QSHWIQZFGQKFMA-UHFFFAOYSA-N
This compound belongs to the class of chemical entities known as aralkylamines. These are alkylamines in which the alkyl group is substituted at one carbon atom by an aromatic hydrocarbyl group.
Chemical entities
Organic compounds
Organic nitrogen compounds
Organonitrogen compounds
Aralkylamines
Aromatic heteromonocyclic compounds
Detected and Quantified
Not Available
Solid
GC-MS Spectrum – GC-MS (4 TMS)splash10-001j-1923000000-70c31b91868484322655View in MoNA
Predicted GC-MS Spectrum – GC-MSNot Available
LC-MS/MS Spectrum – Quattro_QQQ 10V, Positive (Annotated)splash10-0a4i-0090000000-912aa953bdd13d6d18caView in MoNA
LC-MS/MS Spectrum – Quattro_QQQ 25V, Positive (Annotated)splash10-00dl-1900000000-8a87960bb63c4915db22View in MoNA
LC-MS/MS Spectrum – Quattro_QQQ 40V, Positive (Annotated)splash10-006x-9500000000-c934217374e64d6edee3View in MoNA
Predicted LC-MS/MS Spectrum – 10V, Positivesplash10-0a4l-0980000000-423d28b762ba4c07586bView in MoNA
Predicted LC-MS/MS Spectrum – 20V, Positivesplash10-03dl-0910000000-31575764ba8bd8d50279View in MoNA
Predicted LC-MS/MS Spectrum – 40V, Positivesplash10-074r-0900000000-9d96bd59f1a4371a8df4View in MoNA
Predicted LC-MS/MS Spectrum – 10V, Negativesplash10-0059-0790000000-2cc911a67c1aa8e35d99View in MoNA
Predicted LC-MS/MS Spectrum – 20V, Negativesplash10-06ur-0940000000-2c3da60da6ab9523fbf3View in MoNA
Predicted LC-MS/MS Spectrum – 40V, Negativesplash10-0bt9-4900000000-cf52dffb4284f9eeaa5dView in MoNA
Porphyria
- Floderus Y, Sardh E, Moller C, Andersson C, Rejkjaer L, Andersson DE, Harper P: Variations in porphobilinogen and 5-aminolevliinic acid concentrations in plasma and urine from asymptomatic carriers of the acute intermittent porphyria gene with increased porphyrin precursor excretion. Clin Chem. 2006 Apr;52(4):701-7. Epub 2006 Feb 23. [PubMed:16497943 ]
None
Not Available
Not Available
Not Available
Not Available
FDB021916
Not Available
995
C00931
PORPHOBILINOGEN
36404
Porphobilinogen
HMDB00245
HMDB00245
76
1021
PBG
17381
- Dhar GJ, Bossenmaier I, Petryka ZJ, Cardinal R, Watson CJ: Effects of hematin in hepatic porphyria. Further studies. Ann Intern Med. 1975 Jul;83(1):20-30. [PubMed:1147435 ]
- Ivanov E, Pisanets M: Studies on the biosynthesis of porphyrins in erythrocytes after incubation with delta-aminolevulinic acid: an attempt to investigate the pathogenesis of nephrogenic anemia. Acta Biol Med Ger. 1982;41(4):307-13. [PubMed:7124248 ]
- Ellencweig N, Schoenfeld N, Zemishlany Z: Acute intermittent porphyria: psychosis as the only clinical manifestation. Isr J Psychiatry Relat Sci. 2006;43(1):52-6. [PubMed:16910386 ]
- Buchet JP, Lauwerys R, Hassoun A, Dratwa M, Wens R, Collart F, Tielemans C: Effect of aluminum on porphyrin metabolism in hemodialyzed patients. Nephron. 1987;46(4):360-3. [PubMed:3658064 ]
- Tishler PV, Woodward B, OConnor J, Holbrook DA, Seidman LJ, Hallett M, Knighton DJ: High prevalence of intermittent acute porphyria in a psychiatric patient population. Am J Psychiatry. 1985 Dec;142(12):1430-6. [PubMed:4073306 ]
- Hsiao KJ, Lee FY, Wu SJ, Chang WJ: Determination of erythrocyte porphobilinogen deaminase activity using porphobilinogen as substrate. Clin Chim Acta. 1987 Sep 30;168(2):257-8. [PubMed:3677422 ]
- Evans J, Lefkowitch J, Lim CK, Billing B: Fecal porphyrin abnormalities in a patient with features of Rotors syndrome. Gastroenterology. 1981 Dec;81(6):1125-30. [PubMed:7286590 ]
- Sassa S, Solish G, Levere RD, Kappas A: Studies in porphyria. IV. Expression of the gene defect of acute intermittent porphyria in cultured human skin fibroblasts and amniotic cells: prenatal diagnosis of the porphyric trait. J Exp Med. 1975 Sep 1;142(3):722-31. [PubMed:1165472 ]
- Ford RE, Ou CN, Ellefson RD: Assay for erythrocyte uroporphyrinogen I synthase activity, with porphobilinogen as substrate. Clin Chem. 1980 Jul;26(8):1182-5. [PubMed:7389090 ]
- Shiue JW, Lee FY, Hsiao KJ, Tsai YT, Lee SD, Wu SJ: Abnormal thyroid function and hypercholesterolemia in a case of acute intermittent porphyria. Taiwan Yi Xue Hui Za Zhi. 1989 Jul;88(7):729-31. [PubMed:2809566 ]
- Mustajoki P: Normal erythrocyte uroporphyrinogen I synthase in a kindred with acute intermittent porphyria. Ann Intern Med. 1981 Aug;95(2):162-6. [PubMed:7258864 ]
Enzymes
- General function:
- Involved in hydroxymethylbilane synthase activity
- Specific function:
- Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
- Gene Name:
- HMBS
- Uniprot ID:
- P08397
- Molecular weight:
- 39329.74
Reactions
- General function:
- Involved in porphobilinogen synthase activity
- Specific function:
- Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen.
- Gene Name:
- ALAD
- Uniprot ID:
- P13716
- Molecular weight:
- 36294.485