Johnson J L, Rajagopalan K V
J Clin Invest. 1976 Sep;58(3):551-6. doi: 10.1172/JCI108500.
Frozen liver tissue from an individual identified several years ago as sulfite oxidase deficient has been reexamined in light of new knowledge which has been obtained regarding the enzyme. It has been established that hepatic molybdenum levels and xanthine oxidase activity were within normal values and comparable to those observed in control samples preserved from the original study along with the deficient tissue sample. The ability of the patient's liver to synthesize the specific molybdenum cofactor required for activation of de-molybdo sulfite oxidase also appears to have been unimpaired. Using an antibody preparation directed against rat liver sulfite oxidase which also inhibits and precipitates the human enzyme, it has been determined that cross-reacting material with determinants recognized by inhibiting antibodies is absent in the liver sample from the deficient patient. Immunodiffusion experiments gave strong precipitin bands against the control liver extracts, but showed no detectable precipitin reaction between the deficient liver extract and the antibody preparation. The relationship of these findings to a second patient recently identified as sulfite oxidase deficient and to an animal model of the disease are discussed.
根据关于该酶的新知识，对几年前被鉴定为亚硫酸盐氧化酶缺乏的个体的冷冻肝脏组织进行了重新检查。现已确定，肝脏钼水平和黄嘌呤氧化酶活性在正常范围内，与原始研究中保存的对照样本以及缺乏组织样本中观察到的水平相当。患者肝脏合成去钼亚硫酸盐氧化酶激活所需特定钼辅因子的能力似乎也未受损。使用针对大鼠肝脏亚硫酸盐氧化酶的抗体制剂（该制剂也能抑制和沉淀人酶），已确定在来自缺乏患者的肝脏样本中不存在与抑制抗体识别的决定簇发生交叉反应的物质。免疫扩散实验对对照肝脏提取物产生了强沉淀带，但在缺乏肝脏提取物与抗体制剂之间未显示可检测到的沉淀反应。讨论了这些发现与最近鉴定为亚硫酸盐氧化酶缺乏的另一名患者以及该疾病动物模型的关系。
