- 関
- ウロポルフィリノゲンI
WordNet
- the 9th letter of the Roman alphabet (同)i
PrepTutorEJDIC
- 『私は』私が
- iodineの化学記号
UpToDate Contents
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- 1. 急性間欠性ポルフィリン症の臨床症状および診断 clinical manifestations and diagnosis of acute intermittent porphyria
- 2. 急性間欠性ポルフィリン症の病因および発症機序 etiology and pathogenesis of acute intermittent porphyria
- 3. 成人における初回痙攣発作の評価 evaluation of the first seizure in adults
- 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 management of acute intermittent porphyria
English Journal
- Biallelic inactivation of protoporphyrinogen oxidase and hydroxymethylbilane synthase is associated with liver cancer in acute porphyrias.
- Schneider-Yin X1, van Tuyll van Serooskerken AM2, Siegesmund M3, Went P4, Barman-Aksözen J1, Bladergroen RS2, Komminoth P5, Cloots RH6, Winnepenninckx VJ6, Zur Hausen A6, Weber M7, Driessen A6, Poblete-Gutiérrez P8, Bauer P9, Schroeder C9, van Geel M2, Minder EI1, Frank J10.
- Journal of hepatology.J Hepatol.2015 Mar;62(3):734-8. doi: 10.1016/j.jhep.2014.11.029. Epub 2014 Nov 28.
- Variegate porphyria (VP) and acute intermittent porphyria (AIP), the two most common types of acute porphyrias (AHPs), result from a partial deficiency of protoporphyrinogen oxidase (PPOX) and hydroxymethylbilane synthase (HMBS), respectively. A rare but serious complication in the AHPs is hepatocel
- PMID 25445397
- Hydroxymethylbilane Synthase Gene Mutations and Polymorphisms in Brazilian Families with Acute Intermittent Porphyria.
- Gonzaga AD1, de Amorim LM, Fonseca AB, Nogueira TL, Pereira OM, Nagai MA, de Oliveira Barretto OC, Ribeiro GS.
- Annals of human genetics.Ann Hum Genet.2015 Feb 20. doi: 10.1111/ahg.12102. [Epub ahead of print]
- Acute intermittent porphyria (AIP), an autosomal dominant disorder, is caused by a deficiency of hydroxymethylbilane synthase (HMBS). In the present study, we sought to establish a correlation between HMBS activity with the presence of mutations and polymorphisms. Enzyme activity was measured in red
- PMID 25703257
- Acute porphyrias in the USA: features of 108 subjects from porphyrias consortium.
- Bonkovsky HL1, Maddukuri VC2, Yazici C2, Anderson KE3, Bissell DM4, Bloomer JR5, Phillips JD6, Naik H7, Peter I7, Baillargeon G3, Bossi K8, Gandolfo L9, Light C9, Bishop D7, Desnick RJ7.
- The American journal of medicine.Am J Med.2014 Dec;127(12):1233-41. doi: 10.1016/j.amjmed.2014.06.036. Epub 2014 Jul 10.
- BACKGROUND: Recent descriptions of the clinical and laboratory features of subjects with acute porphyrias in the US are lacking. Our aim was to describe clinical, biochemical, and genetic features of 108 subjects.METHODS: Between September 2010 and December 2012, 108 subjects with acute porphyrias (
- PMID 25016127
Japanese Journal
- Purification and Characterization of Human Uroporphyrinogen III Synthase Expressed in Escherichia coli
- Omata Yoshiaki,Sakamoto Hiroshi,Higashimoto Yuichiro [他],HAYASHI Shunsuke,NOGUCHI Masato
- The journal of biochemistry 136(2), 211-220, 2004-08-01
- NAID 10016201529
- 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
- … The side-chain asymmetry of physiological porphyrins is produced by the coopera-tive action of hydroxymethylbilane synthase and uroporphyrinogen (uro'gen) III syn-thase. … Although the role of uro'gen III synthase is essential for the chemistry of por-phyrin biosynthesis, many aspects, structural as well as mechanical, of uro'gen III synthase have yet to be studied. …
- NAID 130003418166
- Human uroporphyrinogen-III synthase : genomic organization, alternative promoters, and erythroid-specific expression
- AIZENCANG G.
- Genomics 70, 223-231, 2000
- NAID 30021497474
Related Links
- uro·por·phy·rin·o·gen I syn·thase (u″ro por″fə rinґə jən sinґthās) hydroxymethylbilane synthase ... Look at other dictionaries: Uroporphyrinogen — Uroporphyrinogens are tetrapyrroles with four propionic acid groups and four acetic acid ...
- Uroporphyrinogen I synthase and uroporphyrinogen III cosynthase Production of uroporphyrin III requires two enzymes. The substrates are four molecules of porphobilinogen. The first reaction is catalyzed by uroporphyrinogen I ...
Related Pictures
★リンクテーブル★
| リンク元 | 「ヘム」 |
| 関連記事 | 「synthase」「I」「Id」「uroporphyrinogen」 |
「ヘム」
- ポルフィリンと鉄イオンの錯体
生合成
| 反応場所 | 酵素 | 補酵素 | 酵素阻害物質 | 酵素の障害による疾患 | ||||||
| ミトコンドリア | サクシニルCoA succinyl-CoA |
グリシン glycine |
5-アミノレブリン酸 aminolevulinic acid aminolevulinate ALA |
CoA | CO2 | 5-アミノレブリン酸シンターゼ ALA synthase |
ビタミンB6 | |||
| 細胞質 | 5-アミノレブリン酸 aminolevulinic acid aminolevulinate ALA |
←2分子 | ポルホビリノゲン porphobilinogen PBG |
2H2O | ALA dehydratase | Pb | ||||
| 細胞質 | ポルホビリノゲン porphobilinogen PBG |
ヒドロキシメチルビラン | 4NH3 | ウロポルフィリゲノンIシンターゼ uroporphyrinogen I synthase |
急性間欠性ポルフィリン症 acute intermittent porphyria | |||||
| 細胞質 | ヒドロキシメチルビラン | ウロポルフィリノゲンIII | ウロポルフィリゲノンIIIシンターゼ uroporphyrinogen III synthase |
|||||||
| 細胞質 | ウロポルフィリノゲンIII | コプロポルフィリノゲンIII | 4CO2 | ウロポルフィリノゲンデカルボキシラーゼ | 晩発性皮膚ポルフィリン症 porphyria cutanea tarda | |||||
| ミトコンドリア | コプロポルフィリノゲンIII | プロトポルフィリノゲンIX | 2CO2 | コプロポルフィリノゲンオキシダーゼ | ||||||
| ミトコンドリア | プロトポルフィリノゲンIX | プロトポルフィリンIX | プロトポルフィリノゲンオキシダーゼ | |||||||
| ミトコンドリア | プロトポルフィリンIX | Fe2+ | ヘム | フェロケラターゼ | Pb | |||||
生合成の場所
- 哺乳類細胞では、赤血球以外は何処でも行われる(ミトコンドリアを有するなら)
- 実際には、赤血球前駆細胞(erythroid precursor cell)で85%行われており、残りは肝細胞で行われている。
「synthase」
- n.
「I」
「Id」
「uroporphyrinogen」
ウロ・ルフィリノーゲン
- 同
- uroporphyrinogens