Tobacman Joanne K
Department of Internal Medicine, University of Iowa Health Care, Iowa City, IA 52242, USA.
Chest. 2003 Jun;123(6):2130-9. doi: 10.1378/chest.123.6.2130.
Cystic fibrosis (CF) is associated with mutation and abnormal function of the cystic fibrosis transmembrane conductance regulator (CFTR) that affects cellular chloride transport. Clinically, CF of the lung is associated with excessive accumulation of secretions, including the sulfated glycosaminoglycans, chondroitin sulfate and dermatan sulfate (DS), both of which contain sulfated N-acetylgalactosamine residues. The sulfatase enzymes, which are a highly conserved group of enzymes with high specificity for designated sulfate groups, include arylsulfatase B, a lysosomal enzyme. Arylsulfatase B, also known as N-acetyl galactosamine 4-sulfatase, can degrade DS and chondroitin-4 sulfate. Previously reported data demonstrated diminished activity of arylsulfatase B in lymphoid cell lines of patients with CF compared to normal control subjects. Frequent infections with Pseudomonas, a sulfatase-producing organism, occur in patients with CF, whereas infections with Mycobacterium tuberculosis, which lacks sulfatase activity, are infrequent. Additional investigation to determine if diminished function of arylsulfatase B is a consistent finding in cells of patients with CF may be informative, and may help to correlate the molecular, biochemical, and clinical characteristics of CF.
囊性纤维化（CF）与囊性纤维化跨膜传导调节因子（CFTR）的突变及功能异常相关，CFTR影响细胞的氯化物转运。临床上，肺部CF与分泌物过度积聚有关，这些分泌物包括硫酸化糖胺聚糖、硫酸软骨素和硫酸皮肤素（DS），二者均含有硫酸化的N-乙酰半乳糖胺残基。硫酸酯酶是一组高度保守的酶，对特定硫酸基团具有高度特异性，其中包括溶酶体酶芳基硫酸酯酶B。芳基硫酸酯酶B也被称为N-乙酰半乳糖胺4-硫酸酯酶，能够降解DS和硫酸软骨素-4。先前报道的数据表明，与正常对照受试者相比，CF患者淋巴细胞系中芳基硫酸酯酶B的活性降低。CF患者常感染能产生硫酸酯酶的铜绿假单胞菌，而缺乏硫酸酯酶活性的结核分枝杆菌感染则较少见。进一步研究以确定芳基硫酸酯酶B功能降低在CF患者细胞中是否为一致发现可能会提供有用信息，并有助于关联CF的分子、生化和临床特征。
