ヒドロキシメチルビラン HMB
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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/07/21 11:46:45」(JST)
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Hydroxymethylbilane
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| Identifiers |
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CAS Number
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73023-76-4 Y |
| ChEBI |
CHEBI:16645 Y |
| ChEMBL |
ChEMBL273676 Y |
| ChemSpider |
767 Y |
| Jmol 3D model |
Interactive image |
| MeSH |
hydroxymethylbilane |
| PubChem |
788 |
InChI
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InChI=1S/C40H46N4O17/c45-17-32-25(12-40(60)61)21(4-8-36(52)53)29(44-32)15-31-24(11-39(58)59)20(3-7-35(50)51)28(43-31)14-30-23(10-38(56)57)19(2-6-34(48)49)27(42-30)13-26-22(9-37(54)55)18(16-41-26)1-5-33(46)47/h16,41-45H,1-15,17H2,(H,46,47)(H,48,49)(H,50,51)(H,52,53)(H,54,55)(H,56,57)(H,58,59)(H,60,61) Y
Key: WDFJYRZCZIUBPR-UHFFFAOYSA-N Y
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InChI=1/C40H46N4O17/c45-17-32-25(12-40(60)61)21(4-8-36(52)53)29(44-32)15-31-24(11-39(58)59)20(3-7-35(50)51)28(43-31)14-30-23(10-38(56)57)19(2-6-34(48)49)27(42-30)13-26-22(9-37(54)55)18(16-41-26)1-5-33(46)47/h16,41-45H,1-15,17H2,(H,46,47)(H,48,49)(H,50,51)(H,52,53)(H,54,55)(H,56,57)(H,58,59)(H,60,61)
Key: WDFJYRZCZIUBPR-UHFFFAOYAI
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SMILES
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O=C(O)Cc1c(cnc1Cc2c(c(c(n2)Cc3nc(c(c3CCC(=O)O)CC(=O)O)Cc4c(c(c(n4)CO)CC(=O)O)CCC(=O)O)CC(=O)O)CCC(=O)O)CCC(=O)O
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| Properties |
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Chemical formula
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C40H46N4O17 |
| Molar mass |
854.81 g/mol |
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).
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| Y verify (what is YN ?) |
| Infobox references |
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Hydroxymethylbilane (HMB), also known as preuroporphyrinogen, is a molecule involved in the metabolism of porphyrin. In the third step, it is generated by the enzyme porphobilinogen deaminase, and in the next step the enzyme uroporphyrinogen III synthase converts it into uroporphyrinogen III.
In general, defects of heme synthesis after formation of HMB lead to photosensitivity.
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Heme metabolic intermediates
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| Porphyrin biosynthesis |
| early mitochondrial: |
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| cytosolic: |
- Porphobilinogen
- Hydroxymethylbilane
- Uroporphyrinogen III
- Coproporphyrinogen III
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| late mitochondrial: |
- Protoporphyrinogen IX
- Protoporphyrin IX
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Heme degradation
and excretion |
| Breakdown of heme |
| spleen: |
- Heme → Biliverdin → Bilirubin
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| blood: |
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| liver: |
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| Intestine, excretion in feces |
- Stercobilinogen
- Stercobilin
- Urobilinogen
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| Kidney, excretion in urine |
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UpToDate Contents
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English Journal
- The effect of long-term -80°C storage of thyroid biospecimens on RNA quality and ensuring fitness for purpose.
- Mathieson W1,2, Betsou F2, Myshunina T3, Pushkarev V3, Pushkarev V3, Shinkarkina A4, Voskoboynyk L3, Thomas GA1.
- Journal of clinical pathology.J Clin Pathol.2016 Dec;69(12):1105-1108. doi: 10.1136/jclinpath-2016-203697. Epub 2016 May 27.
- AIMS: To establish whether RNA degrades in long-term storage at -80°C and whether RNA integrity numbers (RINs) determine 'fitness for purpose' in severely degraded RNA.METHODS: RNA was extracted from 549 thyroid biospecimens stored at -80°C for 0.1-10.9 years then their RINs correlated with stor
- PMID 27235537
- Molecular characterisation of acute intermittent porphyria in a cohort of South African patients and kinetic analysis of two expressed mutants.
- Fortgens P1, Pienaar E2, Corrigall A3, Sonderup M4, Spearman CW4, Meissner P5.
- Journal of clinical pathology.J Clin Pathol.2016 Nov 14. pii: jclinpath-2016-203907. doi: 10.1136/jclinpath-2016-203907. [Epub ahead of print]
- AIMS: Acute intermittent porphyria (AIP) is a disorder of the haem biosynthetic pathway caused by mutations in the hydroxymethylbilane synthase (HMBS) gene. Knowledge of the spectrum of mutations present in South Africa is limited. This study presents the molecular profile of 20 South African patien
- PMID 27849156
- Emerging therapies for acute intermittent porphyria.
- Fontanellas A1, Ávila MA1, Berraondo P2.
- Expert reviews in molecular medicine.Expert Rev Mol Med.2016 Nov 2;18:e17.
- Acute intermittent porphyria (AIP) is an autosomal dominant metabolic disease caused by hepatic deficiency of hydroxymethylbilane synthase (HMBS), the third enzyme of the heme synthesis pathway. The dominant clinical feature is acute neurovisceral attack associated with high production of potentiall
- PMID 27804912
Japanese Journal
- False-Positive Accumulation of Metaiodobenzylguanidine in a Case with Acute Intermittent Porphyria
- Masuda Tomoko,Ota Rie,Ando Takao,Maeda Naoto,Horie Yutaka,Yoshimura Toshiro,Motomura Masakatsu,Kawakami Atsushi
- Internal Medicine 50(9), 1029-1032, 2011
- … A diagnosis of acute intermittent porphyria was finally confirmed based on the characteristic pictures, increased urinary excretion of porphobilinogen, and identification of a heterozygous missense mutation of R173W in the hydroxymethylbilane synthase gene. …
- NAID 130000650107
- Purification and Characterization of Human Uroporphyrinogen III Synthase Expressed in Escherichia coli
- OMATA Yoshiaki,SAKAMOTO Hiroshi,HIGASHIMOTO Yuichiro,HAYASHI Shunsuke,NOGUCHI Masato
- The journal of biochemistry 136(2), 211-220, 2004-08-01
- NAID 10016201529
Related Links
- Hydroxymethylbilane (HMB), also known as preuroporphyrinogen, is a molecule involved in the metabolism of porphyrin. In the third step, it is generated by the enzyme porphobilinogen deaminase, and in the next step the enzyme ...
- References for "Hydroxymethylbilane" online, at universities and in literature... cyclopaedia.net ... Hydroxymethylbilane (HMB), also known as preuroporphyrinogen, is a molecule involved in the metabolism of porphyrin. In the third ...
★リンクテーブル★
[★]
- 英
- hydroxymethylbilane, HMB
- 関
- ビルフィリン症、ヘム
[★]
ヒドロキシメチルビラン hydroxymethylbilane