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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 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.

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1. 先天性骨髄性ポルフィリン症 congenital erythropoietic porphyria
2. 晩発性皮膚ポルフィリン症および肝骨髄性ポルフィリン症：臨床的特徴および診断 porphyria cutanea tarda and hepatoerythropoietic porphyria clinical manifestations and diagnosis
3. 急性間欠性ポルフィリン症の病因、臨床症状および診断 pathogenesis clinical manifestations and diagnosis of acute intermittent porphyria
4. ALA脱水酵素欠損性ポルフィリン症 ala dehydratase porphyria
5. 急性間欠性ポルフィリン症のマネージメントおよび予後 management and prognosis of acute intermittent porphyria

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

Bioscience, Biotechnology, and Biochemistry 72(7), 1908-1914, 2008
NAID 130000029026

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★リンクテーブル★

リンク元	「ヒドロキシメチルビラン」
拡張検索	「uroporphyrinogen III synthase」「uroporphyrinogen III synthase deficiency」「uroporphyrinogen III synthetase」
関連記事	「II」「I」「uroporphyrinogen」

「ヒドロキシメチルビラン」

Uroporphyrinogen III
Identifiers
CAS Registry Number	1976-85-8
MeSH	Uroporphyrinogen+III
PubChem	1179
Properties
Chemical formula	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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	Infobox references

　　[★]

英: hydroxymethylbilane, HMB
関: ビルフィリン症、ヘム

[3]［propphobilinogen, PBG］--([cE2]［porphobilinogen deaminase］)--→[4]［hydroxymethylbilane, HMB］--([cE3]［uroporphyrinogen III synthase］)--→[5]［uroporphyrinogen III］

「uroporphyrinogen III synthase」

　　[★] ウロポルフィリノーゲンIII合成酵素

「uroporphyrinogen III synthase deficiency」

　　[★]

関: uroporphyrinogen III synthase
同: UROS

「uroporphyrinogen III synthetase」