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已发现某些嘌呤补救酶(purine salvage enzy-mes)如腺苷脱氨酶(ADA)、嘌呤核苷酸磷酸化酶(PNP)和5＇-核苷酸酶(5＇-N)与某些代谢的先天缺陷有关.近年有些免疫学家发现ADA和PNP的缺陷分别与儿童的严重联合免疫缺陷病(SLID)和严重T细胞功能失调有关。5＇-N的缺乏与一些不同的原发性免疫缺陷状态如先天性X-连锁的无丙种球蛋白血症(CAG)、慢性淋巴细胞性白血病(CLL)等有关。ADA(E·C·3、5、4、4)催化腺苷和脱氧腺苷脱氨作用,PNP(E·C·2、4、2、
It has been found that certain purine salvage enzymes such as adenosine deaminase (ADA), purine nucleotide phosphorylase (PNP) and 5’-nucleotidase (5’-N) Some metabolic defects in recent years, some immunologists found that ADA and PNP defects and children with severe combined immunodeficiency disease (SLID) and severe T cell dysfunction. The lack of 5’-N is associated with a number of different primary immunodeficiency states, such as congenital X-linked agammaglobulinemia (CAG), chronic lymphocytic leukemia (CLL) and the like. ADA (E · C · 3,5,4,4) catalyzed the deamination of adenosine and deoxyadenosine, PNP (E · C · 2,4,2,