在多种硫酸酯酶缺乏症成纤维细胞中芳基硫酸酯酶A随pH的调节作用
Arysulfatase A modulation with pH in multiple sulfatase deficiency disorder fibroblasts.
作者信息
Fluharty A L, Stevens R L, de la Flor S D, Shapiro L J, Kihara H
出版信息
Am J Hum Genet. 1979 Sep;31(5):574-80.
Abstract
It has been observed that multiple sulfatase deficiency disorder (MSDD) fibroblasts contained from profoundly deficient to near normal amounts of arylsulfatase (ARS) A depending on the medium in which they were cultured. Our present findings show that the major factor determining the enzyme level is the pH of the medium during growth. In media which became acidic or was maintained at low pH (less than 7), the cells expressed the enzymopathy, while in high pH media (7.4), the cells produced enzyme. The high and low enzyme states were reversible. The ARS A deficiency in MSDD must, therefore, be a secondary manifestation of a mutation in another system.
摘要
据观察，多种硫酸酯酶缺乏症（MSDD）成纤维细胞所含芳基硫酸酯酶（ARS）A的量从严重缺乏到接近正常，这取决于它们所培养的培养基。我们目前的研究结果表明，决定酶水平的主要因素是细胞生长期间培养基的pH值。在变酸或维持在低pH值（小于7）的培养基中，细胞表现出酶病，而在高pH值（7.4）的培养基中，细胞产生酶。高酶状态和低酶状态是可逆的。因此，MSDD中ARS A的缺乏必定是另一个系统中突变的次要表现。
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Am J Hum Genet. 1978 May;30(3):249-55.
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