Kihara H, Meek W E, Fluharty A L
Hum Genet. 1986 Sep;74(1):59-62. doi: 10.1007/BF00278786.
It had been shown previously that arylsulfatase A activity was attenuated in pseudo arylsulfatase A deficiency fibroblasts and that subunits of the enzyme were smaller than subunits of the enzyme in normal fibroblasts. Attenuated enzyme activity has now been affirmed in other tissues. Subunits of the enzyme from these sources were also found to be smaller with apparent molecular size 59 and 56 kdaltons. Subunits of enzyme in corresponding control tissues were larger and there was heterogeneity in apparent molecular size as follows: fibroblasts, 63 and 59 kdaltons; liver, 63 and 59 kdaltons; kidney, 63 and 58 kdaltons; spleen, 63 and 58 kdaltons; placenta, 62 and 58 kdaltons; and urine, 61 and 57 kdaltons. Attenuated enzyme activity and structurally altered enzyme in pseudo arylsulfatase A deficiency appears to be systemic. However, the reason for reduced amounts of structurally altered enzyme with normal catalytic activity is unresolved.
先前已表明，在假性芳基硫酸酯酶A缺乏的成纤维细胞中，芳基硫酸酯酶A的活性减弱，且该酶的亚基比正常成纤维细胞中该酶的亚基小。现在已证实在其他组织中酶活性也减弱。还发现来自这些来源的该酶亚基较小，表观分子大小为59和56千道尔顿。相应对照组织中酶的亚基较大，表观分子大小存在异质性，如下：成纤维细胞，63和59千道尔顿；肝脏，63和59千道尔顿；肾脏，63和58千道尔顿；脾脏，63和58千道尔顿；胎盘，62和58千道尔顿；尿液，61和57千道尔顿。假性芳基硫酸酯酶A缺乏时酶活性减弱和酶结构改变似乎是全身性的。然而，具有正常催化活性的结构改变的酶量减少的原因尚未解决。
