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胆色素原

胆色素原
IUPAC名
3-[5-(氨甲基)-4-(羧甲基)-1H-吡咯-3-基]丙酸
识别
CAS号 487-90-1  checkY
PubChem 1021
ChemSpider 995
SMILES
 
  • C1=C(C(=C(N1)CN)CC(=O)O)CCC(=O)O
InChI
 
  • 1/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)
InChIKey QSHWIQZFGQKFMA-UHFFFAOYAF
EINECS 207-666-3
DrugBank DB02272
MeSH Porphobilinogen
性质
化学式 C10H14N2O4
摩尔质量 226.229 g·mol⁻¹
若非注明,所有数据均出自标准状态(25 ℃,100 kPa)下。

胆色素原(英語:Porphobilinogen,亦称为卟胆原,简称为PBG)是一种作为卟啉(Porphyrin)生物合成中间体的吡咯(Pyrrole)衍生物,而卟啉(Porphyrin)為合成血基質的原料之一。

由两分子δ-氨基乙酰丙酸英语Aminolevulinic acid(下圖dALA) 在胆色素原合成酶英语Porphobilinogen synthase(又稱下圖之ALA dehydratase英语Porphobilinogen synthase,圖上ALA dehydrogenase為誤植)催化下缩合、环化而成。
而后4分子胆色素原胆色素原脱氨酶英语Porphobilinogen deaminase(下圖右側 PB deaminase)催化下,将脱去侧链上的氨基,缩聚为含有4个吡咯(Pyrrole)环的羥甲基胆素英语Hydroxymethylbilane。(見下圖右側 Hydroxymethyl bilane)

在急性間歇性紫質症患者之尿液中,胆色素原會有增加的情況[1]

  1. ^ Anderson, Karl E.; Bloomer, Joseph R.; Bonkovsky, Herbert L.; Kushner, James P.; Pierach, Claus A.; Pimstone, Neville R.; Desnick, Robert J. Recommendations for the diagnosis and treatment of the acute porphyrias. Annals of Internal Medicine. 2005-03-15, 142 (6): 439–450 [2016-09-02]. ISSN 1539-3704. PMID 15767622. (原始内容存档于2019-08-13). 
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胆色素原
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