影响脑匀浆中荧光法测定硫酸葡萄糖胺硫酸酯酶（SGSH）活性的变量。
Variables influencing fluorimetric -sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates.
作者信息
Whyte Lauren S, Hopwood John J, Hemsley Kim M, Lau Adeline A
机构信息
Lysosomal Diseases Research Unit, South Australian Health and Medical Research Institute, PO Box 11060, Adelaide, SA 5001, Australia.
出版信息
Mol Genet Metab Rep. 2015 Oct 22;5:60-62. doi: 10.1016/j.ymgmr.2015.10.005. eCollection 2015 Dec.
Deficient -sulfoglucosamine sulfohydrolase (SGSH) enzyme activity causes mucopolysaccharidosis (MPS) type IIIA. A fluorimetric SGSH activity assay is commonly used to examine patient cells. Here, we modified this method for brain homogenates and define the parameters for assay linearity. SGSH activity was suppressed outside of these parameters. This method will enable the accurate measurement of SGSH activity in MPS IIIA tissues to examine disease pathogenesis and evaluate therapies.
磺基葡萄糖胺硫酸酯酶（SGSH）酶活性缺乏会导致ⅢA型黏多糖贮积症（MPS）。荧光法SGSH活性测定通常用于检测患者细胞。在此，我们针对脑匀浆对该方法进行了改进，并确定了测定线性的参数。在这些参数范围之外，SGSH活性受到抑制。该方法将能够准确测量MPS IIIA组织中的SGSH活性，以研究疾病发病机制并评估治疗方法。
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