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- the 9th letter of the Roman alphabet (同)i
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- iodineの化学記号
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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2015/05/06 07:26:41」(JST)
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Uroporphyrinogen III
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Identifiers |
CAS Registry Number
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1976-85-8 Y |
MeSH |
Uroporphyrinogen+III |
PubChem |
1179 |
Properties |
Chemical formula
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C40H44N4O16 |
Molar mass |
836.795 g/mol |
Except where noted otherwise, data is given for materials in their standard state (at 25 °C (77 °F), 100 kPa)
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Y verify (what is: Y/N?) |
Infobox references |
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Uroporphyrinogen III is the first cyclic metabolic intermediate in the biosynthesis of heme. The linear precursor, hydroxymethylbilane is generated from four porphobilinogen (PBG) molecules by the third enzyme in the synthesis, hydroxymethylbilane synthase (or porphobilinogen deaminase). When the linear chain of four PBGs (hydroxymethylbilane) is released from hydroxymethylbilane synthase, the next enzyme in the haem biosynthesis, uroporphyrinogen-III synthase, converts hydroxymethylbilane into the cyclic uroporphyrinogen III. This product is subsequently converted into coproporphyrinogen III by the enzyme uroporphyrinogen III decarboxylase. However, if there is no uroporphyrinogen-III synthase present, the linear tetrapyrrole will be spontaneous cyclised into Uroporphyrinogen I, which is then converted into coproporphyrinogen I, also by uroporphyrinogen III decarboxylase
The difference between the two forms, is the arrangements of the four propionic acid ("P" groups) and the four acetic acid groups ("A" groups), where non-enzyamtically conversion into uroporphyrinogen I results in an AP-AP-AP-AP symmetry, whereas the enzymatically conversion into uroporphyrinogen III lead to a reversed AP-group and hence an AP-AP-AP-PA arrangement.
See also
Heme synthesis—note that some reactions occur in the cytoplasm and some in the mitochondrion (yellow)
Types of tetrapyrroles
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Bilanes
(Linear) |
- Bilirubin
- Biliverdin
- Stercobilinogen
- Stercobilin
- Urobilinogen
- Urobilin
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Phytobilins
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Phycobilins
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- Phycoerythrobilin
- Phycocyanobilin
- Phycourobilin
- Phycoviolobilin
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Macrocycle |
Corrinoids
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- Methylcobalamin
- Adenosylcobalamin
- Cyanocobalamin
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Porphyrins
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Protoporphyrins
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- Protoporphyrin IX
- Heme (b, c, a, o)
- Zinc protoporphyrin
- Magnesium protoporphyrin
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Phytoporphyrins
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- Chlorophyll c
- Protochlorophyllide
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Reduced
porphyrins
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Porphyrinogens
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- Uroporphyrinogen (I, III)
- Coproporphyrinogen (I, III)
- Protoporphyrinogen IX
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Chlorins
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- Chlorophyllide (a, b)
- Chlorophyll (a, b)
- Phaeophytin (a, b)
- Bacteriochlorophyll c
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Bacteriochlorins
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Isobacteriochlorins
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- Siroheme
- Sirohydrochlorin
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Corphins
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Index of biochemical families
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Carbohydrates |
- Alcohols
- Glycoproteins
- Glycosides
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Lipids |
- Eicosanoids
- Fatty acids
- Glycerides
- Phospholipids
- Sphingolipids
- Steroids
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Nucleic acids |
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Proteins |
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Other |
- Tetrapyrroles
- intermediates
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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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Index of cells from bone marrow
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Description |
- Immune system
- Cells
- Physiology
- coagulation
- proteins
- granule contents
- colony-stimulating
- heme and porphyrin
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Disease |
- Red blood cell
- Monocyte and granulocyte
- Neoplasms and cancer
- Histiocytosis
- Symptoms and signs
- Blood tests
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Treatment |
- Transfusion
- Drugs
- thrombosis
- bleeding
- other
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UpToDate Contents
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English Journal
- Advances in understanding the pathogenesis of congenital erythropoietic porphyria.
- Di Pierro E1, Brancaleoni V1, Granata F1.
- British journal of haematology.Br J Haematol.2016 Mar 11. doi: 10.1111/bjh.13978. [Epub ahead of print]
- Congenital erythropoietic porphyria (CEP) is a rare genetic disease resulting from the remarkable deficient activity of uroporphyrinogen III synthase, the fourth enzyme of the haem biosynthetic pathway. This enzyme defect results in overproduction of the non-physiological and pathogenic porphyrin is
- PMID 26969896
- [Congenital erythropoietic porphyria : An update].
- Wenner C1, Neumann NJ1, Frank J2.
- Der Hautarzt; Zeitschrift für Dermatologie, Venerologie, und verwandte Gebiete.Hautarzt.2016 Mar;67(3):216-20. doi: 10.1007/s00105-015-3732-8.
- BACKGROUND: Congenital erythropoetic porphria is a very rare type of autosomal recessive nonacute porphyria. Homozygous or compound heterozygous mutations in the uroporphyrinogen III consynthase gene cause a marked enzymatic deficiency of uroporphyrinogen III consynthase, the fourth enzyme along the
- PMID 26631056
Japanese Journal
- Characterization of a Gene Conferring Red Fluorescence Isolated from an Environmental DNA Library Constructed from Soil Bacteria
- Bioscience, biotechnology, and biochemistry 72(7), 1908-1914, 2008-07-23
- NAID 10027530284
- Characterization of a Gene Conferring Red Fluorescence Isolated from an Environmental DNA Library Constructed from Soil Bacteria
Related Links
- Information about Uroporphyrinogen III in Free online English dictionary. What is Uroporphyrinogen III? Meaning of Uroporphyrinogen III medical term. What does Uroporphyrinogen III mean? Printer Friendly Dictionary, Encyclopedia ...
- El término Uroporphyrinogen III figura en la edición en idioma inglés de Wikipedia. Allí se dice al respecto lo siguiente: Uroporphyrinogen III is the first cyclic metabolic intermediate in the biosynthesis of heme. The linear precursor, hydroxymethylbilane ...
Related Pictures
![Uroporphyrinogen III - Wikipedia, the free](https://1mg.info/0/f/j/0731.jpg)
![Uroporphyrinogen III skeletal.svg](https://1mg.info/0/f/j/0732.jpg)
![Uroporphyrinogen-III-Synthase](https://1mg.info/0/f/j/0733.jpg)
★リンクテーブル★
[★]
- 英
- hydroxymethylbilane, HMB
- 関
- ビルフィリン症、ヘム
[★]
ウロポルフィリノーゲンIII合成酵素
[★]
- 関
- uroporphyrinogen III synthase
- 同
- UROS
[★]
ウロポルフィリノーゲンIII合成酵素
[★]
[★]
[★]
ウロ・ルフィリノーゲン
- 同
- uroporphyrinogens