Study on steroid sulfatase activity in human

人体类固醇硫酸酯酶活性研究

• 批准号: 01570937
• 负责人: MIYAKAWA Isao
• 金额: $ 1.28万
• 依托单位: Oita Medical University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for General Scientific Research (C)
• 财政年份: 1989
• 资助国家: 日本
• 起止时间: 1989 至 1991
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-01570937/
• 关键词: steroid sulfatase; estrogen metabolism; fete-placental function; ichthyosis

MaterialsBlood samples were collected from 7 patients with RXLI(age ; 2 - 22 yr.), 6 carriers of RXLI(32- 48 yr.), and 12 nonpregnant women(22 - 38 yr.), 30 pregnant women(24- 36 yr. ; 1st trimester n=9, 3rd trimester n=21), 15 fetuses(cord blood), 18 male adults(26- 46 yr.), 5 male children(2- 10 yr.)as controls.ResultsSteroid sulfatase activity was extremely lower in patients with RXLI of below 0.005 and 0.079 <plus-minus> 0.018(mean <plus-minus> SD)p mol/mg protein/8 h. in carriers of RXLI. Also, activities in control groups were 0.108 + 0.028 in nonpregnant women, 0.104 <plus-minus> 0.029 in the 1st trimester 0.139 <plus-minus> 0.029 in 3rd trimester of pregnancy, 0.116 <plus-minus> 0.024 in fetuses(umbilical cord blood), 0.088 <plus-minus> 0.014 in male adults and 0.107 <plus-minus> 0.019 p mol/mg protein/8 h. in male children,Steroid sulfatase activity was significantly lower(p<0.001)in patients with RXLI than other groups and significantly greater in the 3rd trimester pregnant women of 0.139 <plus-minus> 0.029 than nonpregnant women of 0.108 <plus-minus> 0.028(P<0.01)and males of 0.088 <plus-minus> 0.014(P<O. 001). Also, sulfatase activity in the 3rd trimester pregnant women was higher(P<0.02)that of feWe found only a small difference(p<0.05)in sulfatase activity in carriers of RXLI and normal females with considerable overtaping between the two groups.Those results suggest that our assay method of steroid sulfatase activity in leucocytes offers a fast and safe method for the diagnosis of placental steroid sulfatase deficiency and the identification of patient with X-linked ichthyosis.

从7名RXLI患者（年龄：2 - 22岁）收集血液样品，6名RXLI携带者（32- 48岁），和12名非妊娠妇女（22 - 38岁），30名孕妇（24- 36岁）孕早期n=9，孕晚期n=21），15例胎儿（脐带血），18例男性成人（26- 46岁），5名男童（2- 10岁）结果RXLI患者的类固醇硫酸酯酶活性极低，分别为0.005和0.079 <plus-minus>± 0.018（平均<plus-minus>SD）pmol/mg蛋白/8h。在RXLI的载体中。此外，对照组中的活性在非妊娠妇女中为0.108 ± 0.028，在妊娠早期为0.104 <plus-minus>± 0.029<plus-minus>，在妊娠晚期为0.139 ± 0.029，<plus-minus>在胎儿（脐带血）中为0.116 ± 0.024，<plus-minus>在男性成人中为0.088 ± 0.014，<plus-minus>在8 h中为0.107 ± 0.019 pmol/mg蛋白质。在男性儿童中，RXLI患者的类固醇硫酸酯酶活性显著低于其他组（P&lt;0.001），妊娠晚期妇女的类固醇硫酸酯酶活性为0.139 ± 0.029，显著高于<plus-minus>非妊娠妇女的类固醇硫酸酯酶活性0.108 <plus-minus>± 0.028（P&lt;0.01）和男性的类固醇硫酸酯酶活性0.088 <plus-minus>± 0.014（P&lt;0.01）。001）。还有，妊娠晚期妇女硫酸酯酶活性较高，（P&lt;0.02）两组间差异无显著性（P&gt; 0.05（p&lt;0.05），但不影响正常人的生活。RXLI携带者与正常妇女血清中硫酸酯酶活性有显著性差异，提示本研究建立的白细胞硫酸酯酶活性检测方法为胎盘硫酸酯酶缺乏症的诊断提供了一种快速、安全的方法。X连锁鱼鳞病患者鉴定