Kamoun P
Laboratoire de Biochimie MéDdicale B, Hôpital Necker Enfants Malades, Paris, France.
Amino Acids. 2004 Jun;26(3):243-54. doi: 10.1007/s00726-004-0072-x. Epub 2004 Apr 8.
Hydrogen sulfide is one of three gases involved in biological functions and synthesized in vivo. Like NO and CO, it seems to act as a neuromodulator: it modulates NMDA glutamate receptor function. CBS seems to be the only source of hydrogen sulfide in the brain, whereas the liver synthesizes hydrogen sulfide via cystathionase. In the heart, the third pathway for the hydrogen sulfide synthesis, the 3-mercaptopyruvate pathway is used. Only two diseases characterized by alterations of hydrogen sulfide metabolism have been described: decreased hydrogen sulfide synthesis in the brains of Alzheimer's disease patients and increased hydrogen sulfide synthesis due to the overexpression of CBS in Down syndrome patients.
硫化氢是参与生物功能且在体内合成的三种气体之一。与一氧化氮和一氧化碳一样,它似乎起着神经调节剂的作用:调节NMDA谷氨酸受体功能。胱硫醚β-合成酶似乎是大脑中硫化氢的唯一来源,而肝脏通过胱硫醚酶合成硫化氢。在心脏中,使用硫化氢合成的第三条途径,即3-巯基丙酮酸途径。仅描述了两种以硫化氢代谢改变为特征的疾病:阿尔茨海默病患者大脑中硫化氢合成减少,以及唐氏综合征患者中由于胱硫醚β-合成酶过表达导致硫化氢合成增加。