创建日期
2005-11-16 15:48:42 UTC
更新日期
2020-10-09 21:08:42 UTC
描述
Porphobilinogen (PBG) is a pyrrole-containing intermediate in the biosynthesis of porphyrins. It is generated from aminolevulinate (ALA) by the enzyme ALA dehydratase. Porphobilinogen is then converted into hydroxymethylbilane by the enzyme porphobilinogen deaminase (also known as hydroxymethylbilane synthase). Under certain conditions, porphobilinogen can act as a phototoxin, a neurotoxin, and a metabotoxin. A phototoxin leads to cell damage upon exposure to light. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of porphyrins are associated with porphyrias such as porphyria variegate, acute intermittent porphyria, and hereditary coproporphyria (HCP). There are several types of porphyrias (most are inherited). Hepatic porphyrias are characterized by acute neurological attacks (seizures, psychosis, extreme back and abdominal pain, and an acute polyneuropathy), while the erythropoietic forms present with skin problems (usually a light-sensitive blistering rash and increased hair growth). The neurotoxicity of porphyrins may be due to their selective interactions with tubulin, which disrupt microtubule formation and cause neural malformations (PMID: 3441503).
同义词
1:5-(Aminomethyl)-4-(carboxymethyl)-pyrrole-3-propionate
2:5-(Aminomethyl)-4-(carboxymethyl)-pyrrole-3-propionic acid
3:PBG
4:Porphobilinogen
化学名称
3-[5-(aminomethyl)-4-(carboxymethyl)-1H-pyrrol-3-yl]propanoic acid
代谢物结构字符串
NCC1=C(CC(O)=O)C(CCC(O)=O)=CN1
inchi标识符
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)
印记键
QSHWIQZFGQKFMA-UHFFFAOYSA-N
分类学
description: belongs to the class of organic compounds known as aralkylamines. These are alkylamines in which the alkyl group is substituted at one carbon atom by an aromatic hydrocarbyl group.
description:Aralkylamines
kingdom:Organic compounds
super_class:Organic nitrogen compounds
class:Organonitrogen compounds
sub_class:Amines
molecular_framework:Aromatic heteromonocyclic compounds
1:Amino acids
2:Azacyclic compounds
3:Carbonyl compounds
4:Carboxylic acids
5:Dicarboxylic acids and derivatives
6:Heteroaromatic compounds
7:Hydrocarbon derivatives
8:Monoalkylamines
9:Organic oxides
10:Organopnictogen compounds
11:Substituted pyrroles
12:Amino acid
13:Amino acid or derivatives
14:Aralkylamine
15:Aromatic heteromonocyclic compound
16:Azacycle
17:Carbonyl group
18:Carboxylic acid
19:Carboxylic acid derivative
20:Dicarboxylic acid or derivatives
21:Heteroaromatic compound
22:Hydrocarbon derivative
23:Organic oxide
24:Organic oxygen compound
25:Organoheterocyclic compound
26:Organooxygen compound
27:Organopnictogen compound
28:Primary aliphatic amine
29:Primary amine
30:Pyrrole
31:Substituted pyrrole
32:aralkylamino compound
33:dicarboxylic acid
34:pyrroles
本体论
term:Physiological effect
definition:The effect on an organism physiology, resulting from its exposure to a chemical.
parent_id:
level:1
type:parent
term:Health effect
definition:A health condition or observation associated with a stimuli or with a biological activity of a chemical.
parent_id:7693
*level:2
type:parent
term:Health condition
definition:A health effect that consists on short or long-term disease, condition, disorder, syndrome or constant abnormality.
parent_id:7694
**level:3
type:parent
term:Porphyria
definition:An inherited metabolic disorder that involves certain enzymes in the heme bio-synthetic pathway resulting in the overproduction and accumulation of the porphyrins. (do).
parent_id:7695
***level:4
type:child
term:Hereditary coproporphyria
definition:
parent_id:7695
***level:4
type:child
synonym:Hcp
term:Disposition
definition:A concept that describes the origin of a chemical, its location within an organism, or its route of exposure.
parent_id:
level:1
type:parent
term:Route of exposure
definition:A mean by which a chemical agent comes in contact with an organism, either under intended or unintended circumstances.
parent_id:7724
*level:2
type:parent
term:Enteral
definition:Chemical exposure via the alimentary canal (mouth to anus).
parent_id:7743
**level:3
type:parent
term:Ingestion
definition:Chemical exposure facilitated by entry through the mouth.
parent_id:7744
***level:4
type:child
synonym:Digestion
term:Source
definition:Natural or synthetic origin of a chemical.
parent_id:7724
*level:2
type:parent
term:Endogenous
definition:
parent_id:7735
**level:3
type:child
term:Food
definition:
parent_id:7735
**level:3
type:child
term:Biological
definition:A living organism (species or a higher taxonomy rank), in which a chemical can be found.
parent_id:7735
**level:3
type:parent
term:Animal
definition:A living organism belonging to the kingdom animalia. it feeds on organic matter, typically having specialized sense organs and nervous system and able to respond rapidly to stimuli.
parent_id:7736
***level:4
type:child
synonym:Fauna
term:Plant
definition:A living organism belonging to the kingdom plantea. typically, it grows in a permanent site, absorbs water and inorganic substances through its roots, and synthesizes nutrients in its leaves by photosynthesis using the green pigment chlorophyll. examples incude trees, shrubs, herbs, grasses, ferns, and mosses.
parent_id:7736
***level:4
type:parent
synonym:Flora
term:Biological location
definition:The physiological origin within an organism, including anatomical compnents, biofluids and excreta.
parent_id:7724
*level:2
type:parent
term:Organ and components
definition:An anatomical organizational level including multiple tissues or substructures, which enables a common biological function.
parent_id:7725
**level:3
type:parent
term:Liver
definition:An abdominal organ that has variable lobation which are composed mainly of hepatic lobules. (nci).
parent_id:7727
***level:4
type:child
term:Biofluid and excreta
definition:A liquid, semi-solid or solid material originating in the body.
parent_id:7725
**level:3
type:parent
term:Urine
definition:Excretion in liquid state processed by the kidney.
parent_id:7731
***level:4
type:child
term:Blood
definition:A liquid tissue with the primary function of transporting oxygen and carbon dioxide (nci). it supplies the tissues with nutrients, removes waste products, and contains various components of the immune system defending the body against infection.
parent_id:7731
***level:4
type:child
term:Subcellular
definition:An anatomical organizational level including a component within a biological cell .
parent_id:7725
**level:3
type:parent
term:Cytoplasm
definition:The portion of the cell contained within the plasma membrane but excluding the nucleus.
parent_id:7730
***level:4
type:child
synonym:Cytoplasma
term:Process
definition:Biological or chemical events, or a series thereof, leading to a known function or end-product.
parent_id:
level:1
type:parent
term:Naturally occurring process
definition:Naturally-occurring molecular events or a series thereof, leading to a known function or end-product.
parent_id:7659
*level:2
type:parent
term:Biological process
definition:Biological or chemical events or a series thereof, leading to a known function or end-product within an organism.
parent_id:7660
**level:3
type:parent
term:Biochemical pathway
definition:A linked series of chemical reactions that occur in a defined order within or between organism cells, and lead to a known function or end product.
parent_id:7661
***level:4
type:parent
term:Role
definition:The purpose or function assumed by a chemical, either naturally or as intended by humans .
parent_id:
level:1
type:parent
term:Indirect biological role
definition:An indirect function of a chemical which affect the physiological state of any organism (apart from the source organism, if of biological source).
parent_id:7671
*level:2
type:parent
term:Neurotoxin
definition:A chemical substance that interferes with the normal functioning of the nervous system.
parent_id:7691
**level:3
type:child
synonym:Neurotoxic
synonym:Neurotoxic agent
term:Phototoxin
definition:A chemical substance that leads to cell damage upon exposure to light.
parent_id:7691
**level:3
type:child
term:Metabotoxin
definition:An endogenous metabolite that causes adverse health effects at chronically high levels.
parent_id:7691
**level:3
type:child
term:Biological role
definition:The biological function of a chemical. the biological role answers the question how a chemical is involved in molecular processes in an organism. this can include biochemical effects of non-endogenous chemicals, which are also assigned an industrial application such as pharmaceuticals. the biological role is limited to cellular levls, and will not include role at system process level, such as a chemical which has a role in a disease.
parent_id:7671
*level:2
type:parent
term:Metabolite
definition:A chemical substance produced during metabolism process, as an intermediate or end-product.
parent_id:7672
**level:3
type:child
term:Industrial application
definition:The assumed function of a chemical utilized by human.
parent_id:7671
*level:2
type:parent
term:Pharmaceutical industry
definition:A pharmacologic activity for which a chemical substance is utilized owing to its biological role.
parent_id:7678
**level:3
type:parent
term:Pharmaceutical
definition:
parent_id:7679
***level:4
type:child
预测性质
1:
kind:logp
value:-2.40
source:ALOGPS
2:
kind:logs
value:-1.92
source:ALOGPS
3:
kind:solubility
value:2.72 g/L
source:ALOGPS
4:
kind:logp
value:-2.7
source:ChemAxon
5:
kind:pka_strongest_acidic
value:3.66
source:ChemAxon
6:
kind:pka_strongest_basic
value:8.78
source:ChemAxon
7:
kind:iupac
value:3-[5-(aminomethyl)-4-(carboxymethyl)-1H-pyrrol-3-yl]propanoic acid
source:ChemAxon
8:
kind:average_mass
value:226.2292
source:ChemAxon
9:
kind:mono_mass
value:226.095356946
source:ChemAxon
10:
kind:smiles
value:NCC1=C(CC(O)=O)C(CCC(O)=O)=CN1
source:ChemAxon
11:
kind:formula
value:C10H14N2O4
source:ChemAxon
12:
kind:inchi
value: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)
source:ChemAxon
13:
kind:inchikey
value:QSHWIQZFGQKFMA-UHFFFAOYSA-N
source:ChemAxon
14:
kind:polar_surface_area
value:116.41
source:ChemAxon
15:
kind:refractivity
value:56.38
source:ChemAxon
16:
kind:polarizability
value:22.56
source:ChemAxon
17:
kind:rotatable_bond_count
value:6
source:ChemAxon
18:
kind:acceptor_count
value:5
source:ChemAxon
19:
kind:donor_count
value:4
source:ChemAxon
20:
kind:physiological_charge
value:-1
source:ChemAxon
21:
kind:formal_charge
value:0
source:ChemAxon
22:
kind:number_of_rings
value:1
source:ChemAxon
23:
kind:bioavailability
value:Yes
source:ChemAxon
24:
kind:rule_of_five
value:Yes
source:ChemAxon
25:
kind:ghose_filter
value:No
source:ChemAxon
26:
kind:veber_rule
value:No
source:ChemAxon
27:
kind:mddr_like_rule
value:No
source:ChemAxon
生物学性质
1:
cellular:(1):Cytoplasm
2:
biospecimen:(1):Blood
biospecimen:(2):Urine
3:
tissue:(1):Erythrocyte
tissue:(2):Liver
4:
[1]:
name:Acute Intermittent Porphyria
smpdb_id:SMP00344
kegg_map_id:
[2]:
name:Congenital Erythropoietic Porphyria (CEP) or Gunther Disease
smpdb_id:SMP00345
kegg_map_id:
[3]:
name:Hereditary Coproporphyria (HCP)
smpdb_id:SMP00342
kegg_map_id:
[4]:
name:Porphyria Variegata (PV)
smpdb_id:SMP00346
kegg_map_id:
[5]:
name:Porphyrin Metabolism
smpdb_id:SMP00024
kegg_map_id:map00860
正常浓度
1:
biospecimen:Blood
concentration_value:0.06 (0.00-0.12)
concentration_units:uM
subject_age:Adult (>18 years old)
subject_sex:Both
subject_condition:Normal
2:
biospecimen:Urine
concentration_value:0.31 +/- 0.13
concentration_units:umol/mmol creatinine
subject_age:Children (1-13 years old)
subject_sex:Both
subject_condition:Normal
3:
biospecimen:Urine
concentration_value:0.29 +/- 0.14
concentration_units:umol/mmol creatinine
subject_age:Adult (>18 years old)
subject_sex:Both
subject_condition:Normal
4:
biospecimen:Urine
concentration_value:0-1.5
concentration_units:umol/mmol creatinine
subject_age:Adult (>18 years old)
subject_sex:Both
subject_condition:Normal
5:
biospecimen:Urine
concentration_value:<=0.579
concentration_units:umol/mmol creatinine
subject_age:Adult (>18 years old)
subject_sex:Both
subject_condition:Normal
异常浓度
1:
biospecimen:Blood
concentration_value:3.1 +/- 1.0
concentration_units:uM
patient_age:Adult (>18 years old)
patient_sex:Both
patient_information:Porphyria
2:
biospecimen:Urine
concentration_value:57.1 (19.6-132.7)
concentration_units:umol/mmol creatinine
patient_age:Adult (>18 years old)
patient_sex:Both
patient_information:Acute intermittent porphyria
3:
biospecimen:Urine
concentration_value:17.2 (74.9-41.6)
concentration_units:umol/mmol creatinine
patient_age:Adult (>18 years old)
patient_sex:Both
patient_information:Acute intermittent porphyria
4:
biospecimen:Urine
concentration_value:9.7 (21.8-10.5)
concentration_units:umol/mmol creatinine
patient_age:Adult (>18 years old)
patient_sex:Both
patient_information:Acute intermittent porphyria
5:
biospecimen:Urine
concentration_value:2.3 (5.1-2.9)
concentration_units:umol/mmol creatinine
patient_age:Adult (>18 years old)
patient_sex:Both
patient_information:Asymptomatic acute intermittent porphyria
6:
biospecimen:Urine
concentration_value:0.589-0.654
concentration_units:umol/mmol creatinine
patient_age:Adult (>18 years old)
patient_sex:Male
patient_information:Protoporphyria, Erythropoietic
疾病参考
1:
name:Acute intermittent porphyria
omim_id:176000
[1]:
reference_text:Zuijderhoudt FM, Koehorst SG, Kluitenberg WE, Dorresteijn-de Bok J: On accuracy and precision of a HPLC method for measurement of urine porphyrin concentrations. Clin Chem Lab Med. 2000 Mar;38(3):227-30.
pubmed_id:10905759
[2]:
reference_text:Marsden JT, Rees DC: Urinary excretion of porphyrins, porphobilinogen and delta-aminolaevulinic acid following an attack of acute intermittent porphyria. J Clin Pathol. 2014 Jan;67(1):60-5. doi: 10.1136/jclinpath-2012-201367. Epub 2013 Aug 1.
pubmed_id:23908454
2:
name:Porphyria
omim_id:
[1]:
reference_text:Gorchein A, Guo R, Lim CK, Raimundo A, Pullon HW, Bellingham AJ: Porphyrins in urine, plasma, erythrocytes, bile and faeces in a case of congenital erythropoietic porphyria (Gunther's disease) treated with blood transfusion and iron chelation: lack of benefit from oral charcoal. Biomed Chromatogr. 1998 Nov-Dec;12(6):350-6.
pubmed_id:9861496
[2]:
reference_text:Floderus Y, Sardh E, Moller C, Andersson C, Rejkjaer L, Andersson DE, Harper P: Variations in porphobilinogen and 5-aminolevulinic 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_id:16497943
[3]:
reference_text:Hindmarsh JT, Oliveras L, Greenway DC: Biochemical differentiation of the porphyrias. Clin Biochem. 1999 Nov;32(8):609-19.
pubmed_id:10638943
3:
name:Protoporphyria, Erythropoietic
omim_id:177000
[1]:
reference_text:MAGNUS IA, JARRETT A, PRANKERD TA, RIMINGTON C: Erythropoietic protoporphyria. A new porphyria syndrome with solar urticaria due to protoporphyrinaemia. Lancet. 1961 Aug 26;2(7200):448-51.
pubmed_id:13765301
代谢途径的数据库编号
PORPHOBILINOGEN
综合参考
Frydman, Benjamin; Despuy, Maria E.; Rapoport, Henry. Pyrroles from azaindoles. A synthesis of porphobilinogen. Journal of the American Chemical Society (1965), 87(15), 3530-1.
一般参考
1:
protein_accession:HMDBP00358
name:Porphobilinogen deaminase
uniprot_id:P08397
gene_name:HMBS
protein_type:Unknown
2:
protein_accession:HMDBP01060
name:Delta-aminolevulinic acid dehydratase
uniprot_id:P13716
gene_name:ALAD
protein_type:Unknown
蛋白质结合
1:
reference_text: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_id:1147435
2:
reference_text: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_id:7124248
3:
reference_text: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_id:16910386
4:
reference_text: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_id:3658064
5:
reference_text:Tishler PV, Woodward B, O'Connor 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_id:4073306
6:
reference_text: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_id:3677422
7:
reference_text:Evans J, Lefkowitch J, Lim CK, Billing B: Fecal porphyrin abnormalities in a patient with features of Rotor's syndrome. Gastroenterology. 1981 Dec;81(6):1125-30.
pubmed_id:7286590
8:
reference_text: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_id:1165472
9:
reference_text: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_id:7389090
10:
reference_text: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_id:2809566
11:
reference_text:Mustajoki P: Normal erythrocyte uroporphyrinogen I synthase in a kindred with acute intermittent porphyria. Ann Intern Med. 1981 Aug;95(2):162-6.
pubmed_id:7258864
12:
reference_text:Winkelman JW, Collins GH: Neurotoxicity of tetraphenylporphinesulfonate TPPS4 and its relation to photodynamic therapy. Photochem Photobiol. 1987 Nov;46(5):801-7.
pubmed_id:3441503