Uroporphyrinogen I
Uroporphyrinogen I
Names
IUPAC name
3,3′,3′′,3′′′-[3,8,13,18-Tetrakis(carboxymethyl)-10,15,20,22,23,24-hexahydro-5H ,21H -porphyrin-2,7,12,17-tetrayl]tetrapropanoic acid
Systematic IUPAC name
3,3′,3′′,3′′′-[14 ,33 ,53 ,73 -Tetrakis(carboxymethyl)-11 H ,31 H ,51 H ,71 H -1,3,5,7(2,5)-tetrapyrrolacyclooctaphane-13 ,34 ,54 ,74 -tetrayl]tetrapropanoic acid
Identifiers
ChemSpider
InChI=1S/C40H44N4O16/c45-33(46)5-1-17-21(9-37(53)54)29-14-26-19(3-7-35(49)50)23(11-39(57)58)31(43-26)16-28-20(4-8-36(51)52)24(12-40(59)60)32(44-28)15-27-18(2-6-34(47)48)22(10-38(55)56)30(42-27)13-25(17)41-29/h41-44H,1-16H2,(H,45,46)(H,47,48)(H,49,50)(H,51,52)(H,53,54)(H,55,56)(H,57,58)(H,59,60)
N Key: QTTNOSKSLATGQB-UHFFFAOYSA-N
N InChI=1/C40H44N4O16/c45-33(46)5-1-17-21(9-37(53)54)29-14-26-19(3-7-35(49)50)23(11-39(57)58)31(43-26)16-28-20(4-8-36(51)52)24(12-40(59)60)32(44-28)15-27-18(2-6-34(47)48)22(10-38(55)56)30(42-27)13-25(17)41-29/h41-44H,1-16H2,(H,45,46)(H,47,48)(H,49,50)(H,51,52)(H,53,54)(H,55,56)(H,57,58)(H,59,60)
Key: QTTNOSKSLATGQB-UHFFFAOYAX
O=C(O)CCc1c(c5[nH]c1Cc2[nH]c(c(c2CC(=O)O)CCC(=O)O)Cc3c(c(c([nH]3)Cc4c(c(c([nH]4)C5)CCC(=O)O)CC(=O)O)CCC(=O)O)CC(=O)O)CC(=O)O
Properties
C 40 H 44 N 4 O 16
Molar mass
836.804 g·mol−1
Except where otherwise noted, data are given for materials in their
standard state (at 25 °C [77 °F], 100 kPa).
Chemical compound
Uroporphyrinogen I is an isomer of uroporphyrinogen III , a metabolic intermediate in the biosynthesis of heme . A type of porphyria is caused by production of uroporphyrinogen I instead of III.
In living organisms, uroporphyrinogen I occurs as a side branch of the main porphyrin synthesis pathway. In the normal pathway, the linear tetrapyrrole precursor preuroporphyrinogen (a substituted hydroxymethylbilane ) is converted by the enzyme uroporphyrinogen-III cosynthase into the cyclic uroporphyrinogen III ; which is then converted to coproporphyrinogen III on the way to porphyrins like heme . Uroporphyrinogen I is instead produced spontaneously from preuroporphyrinogen when the enzyme is not present.[1] [2]
Uroporphyrinogen I synthesis from preuroporphyrinogen
The difference between the I and III forms is the arrangement of the four carboxyethyl groups (propionic acid , "P") and the four carboxymethyl groups (acetic acid , "A"). The non-enzymatic conversion to uroporphyrinogen I results in the sequence AP-AP-AP-AP , whereas the enzymatic conversion into uroporphyrinogen III leads to reversal of one AP-group and hence an AP-AP-AP-PA arrangement.
If synthesized, uroporphyrinogen I is then converted into coproporphyrinogen I by the same enzyme (uroporphyrinogen decarboxylase ) that acts on the III form; but that product, which is cytotoxic , then accumulates causing the pathology congenital erythropoietic porphyria .[2] [3]
References