Cagianut B, Schnebli H P, Rhyner K, Furrer J
Klin Wochenschr. 1984 Sep 17;62(18):850-4. doi: 10.1007/BF01712000.
In mammals the major portion of cyanide is converted to thiocyanate by the liver enzyme thiosulfate sulfur transferase (TST) (rhodanese). We have found a much reduced activity of this enzyme in liver biopsies from two affected males of a family with Leber's hereditary optic atrophy and in two isolated cases of the same disease, (compared to liver biopsies from controls or liver samples obtained at autopsy). In one of the patients we studied the effect of a 3-day thiosulfate infusion. The urinary excretion of thiocyanate which was low prior to the infusion was raised during the thiosulfate treatment; in a healthy control person the same thiosulfate infusion did not alter the thiocyanate excretion rate. This suggests that cyanide detoxification which is suboptimal in patients with Leber's disease may be increased by thiosulfate infusion.
在哺乳动物中，大部分氰化物会被肝脏中的硫代硫酸盐硫转移酶（TST）（硫氰酸酶）转化为硫氰酸盐。我们发现，在一个患有Leber遗传性视神经萎缩的家族中，两名患病男性的肝脏活检样本以及另外两例孤立的同病患者中，这种酶的活性大幅降低（与对照组的肝脏活检样本或尸检获得的肝脏样本相比）。在我们研究的一名患者中，我们观察了为期3天的硫代硫酸盐输注的效果。输注前硫氰酸盐的尿排泄量较低，在硫代硫酸盐治疗期间有所升高；而在一名健康对照者中，相同的硫代硫酸盐输注并未改变硫氰酸盐的排泄率。这表明，硫代硫酸盐输注可能会提高Leber病患者体内次优的氰化物解毒能力。
