旋磁场对大鼠血清铜蓝蛋白活力的影响

用联大茴香胺法测定大白鼠血清中铜蓝蛋白的活力，大白鼠体外血清在旋磁场中曝磁３０分钟后，血清铜蓝蛋白活力增加，而大白鼠整体血清在旋磁场中曝磁３０分钟后，血清铜蓝蛋白活力无明显变化。     

Vaccination against meningococcus in complement-deficient individuals

Autoantibodies to CRP were reported previously in patients suffering from toxic oil syndrome. This syndrome resembles autoimmune diseases such as systemic lupus erythematosus (SLE) or systemic scleroderma. We therefore examined the prevalence of antibodies to CRP and other acute-phase proteins in autoimmune diseases, including SLE, subacute cutaneous lupus erythematosus (SCLE), systemic scleroderma (SSc), and primary biliary cirrhosis (PBC), as well as in bone marrow transplantation-induced chronic graft-versus-host disease and eosinophilia–myalgia syndrome. IgG antibodies to CRP were found in 78% of SLE and in 30% of SCLE patients, while 16% of patients with PBC were positive. In up to 45% of patients with SSc predominantly IgG antibodies to ceruloplasmin were detectable. Lack of systemic involvement as in discoid lupus erythematosus and localized scleroderma (morphea) correlated with low or absent antibody formation. However, no correlation was found between anti-acute-phase protein antibodies with liver disease or other organ involvement. Adsorption studies revealed that non-native epitopes on the CRP molecule, termed modified CRP, are the main target of antibodies. Chronic inflammatory tissue injury in systemic autoimmune disease might increase the presentation of cryptic epitopes of CRP to the threshold required for T cell activation.     

Biological functions of ceruloplasmin and their deficiency caused by mutation in genes regulating copper and iron metabolism

Ceruloplasmin, a multicopper ferroxidase, is involved in iron and copper homeostasis and integrates these metabolic pathways. Impaired biosynthesis of ceruloplasmin caused by gene mutations disturbs iron metabolism with iron deposition in different organs, especially in the basal ganglia, and severe neuronal damage. Dysfunction of ATP7B, a copper-transporting ATPase leads to the development of Wilson’s disease,i.e., multiple abnormalities in copper metabolism associated with reduced synthesis of holoceruloplasmin and biliary copper excretion controlled by both proteins. The lowest content of serum ceruloplasmin is observed in the most grave early neurological form of Wilson’s disease (according to N. V. Konovalov’s classification), which confirms the important role of ceruloplasmin in the striatal metabolism of catecholamines. Translated fromByulleten’ Eksperimental’noi Biologii i Meditsiny, Vol. 130, No. 8, pp. 124–133, August, 2000     

消化系统肿瘤患者血清铜蓝蛋白氧化酶活力的变化

目的：探讨消化系统肿瘤患者血清蓝蛋白活力的变化。方法：以Ｏ－ｄｉａｎｉｓｉｄｉｎ为基质,采用联大茴香胺酶比色法检测８８例消化系统患者和８５名健康人血清铜蓝蛋白的活力。结果：消化系统肿瘤患者ＳＣＰ活力均高于健康人,而食管癌、贲门癌、直肠癌及肝癌患者之间ＳＣＰ活力差异均无显著性。结论：ＳＣＰ对协助诊断和筛选消化系统肿瘤有参考价值。     

大鼠海马内注射β淀粉样蛋白1-40抑制铜蓝蛋白表达

目的:通过检测阿尔茨海默病(Alzheimer disease,AD)模型大鼠海马铜蓝蛋白(glycan-phosphatidyli-nositol ceruloplasmin,GPI-Cp)的变化,探讨AD时脑铁增高的机制。方法:取雄性(290±10)g SD大鼠,经海马内注射(amyloid beta-peptide 1-40,Aβ1-40)建立AD模型,于1、2、3和4周取各组大鼠脑组织,分离海马,用RT-PCR检测GPI-Cp mRNA表达情况,免疫组织化学染色检测GPI-Cp蛋白表达情况。结果:海马的星形胶质细胞、血管内皮细胞、室管膜细胞均有GPI-Cp的mRNA和蛋白表达;而海马的锥体细胞仅轻度表达,颗粒细胞不表达。注射Aβ1-40后,模型组海马GPI-Cp mRNA和蛋白表达均随着时间延长逐渐降低,具有统计学意义(P<0.01)。结论:海马内注射Aβ1-40可引起大鼠海马GPI-Cp表达减少,GPI-Cp表达减少可能是AD时脑铁增高的原因。     

重症肺炎患儿血清PCT、hs-CRP、CER水平与心肌损害发生率的关系探讨

目的检测重症肺炎患儿血清降钙素原(PCT)、超敏C反应蛋白(hs-CRP)、铜蓝蛋白(CER)水平,并分析其与心肌损害发生率的关系。方法对医院2015年4月~2018年9月收治的212例重症肺炎患儿的资料进行回顾,检测其基线血清PCT、hs-CRP和CER水平。计算心肌损害发生率,对比心肌损害发生者和未发生者基线血清PCT、hs-CRP和CER水平。采用Logistic回归分析探讨上述血清因子与重症肺炎患儿心肌损害的关系,并绘制受试者工作特征曲线(ROC)探讨基线血清PCT、hs-CRP、CER水平及三者联合对心肌损害的预测效能。结果本组患儿心肌损害发生率为11.79%,发生时间为(7.15±2.08)d;心肌损害发生者基线血清PCT、hs-CRP和CER水平均高于未发生组(P<0.05);发生者年龄<3岁、并发呼吸衰竭、低蛋白血症、低氧血症、低钾血症、发病至入院时间>7d、发病至入院时间3~7d、基线PCT升高>50%、基线hs-CRP升高>50%、基线CER升高>50%构成比均高于未发生者(P<0.05),经Logistic回归分析证实均为心肌损害的危险因素;基线PCT、hs-CRP、CER水平预测心肌损害的Cut-off值分别为8.45pg/L、12.50mg/mL、402.47mg/L,三者联合预测心肌损害的灵敏度、特异度、AUC分别为92.00%、97.86%、0.896,均高于单独预测。结论重症肺炎患儿并心肌损害发生风险高,发生者基线PCT、hs-CRP、CER水平较高,年龄<3岁、并发呼吸衰竭等均是其危险因素,且基线PCT、hs-CRP、CER水平联合预测心肌损害的效能较高。     

Ceruloplasmin gene‐deficient mice with experimental autoimmune encephalomyelitis show attenuated early disease evolution

We conducted a microarray study to identify genes that are differentially regulated in the spinal cords of mice with the inflammatory disease experimental autoimmune encephalomyelitis (EAE) relative to healthy mice. In total 181 genes with at least a two‐fold increase in expression were identified, and most of these genes were associated with immune function. Unexpectedly, ceruloplasmin (Cp), a ferroxidase that converts toxic ferrous iron to its nontoxic ferric form and also promotes the efflux of iron from astrocytes in the CNS, was shown to be highly upregulated (13.2‐fold increase) in EAE spinal cord. Expression of Cp protein is known to be increased in several neurological conditions, but the role of Cp regulation in CNS autoimmune disease is not known. To investigate this, we induced EAE in Cp gene knockout, heterozygous, and wild‐type mice. Cp knockout mice were found to have slower disease evolution than wild‐type mice (EAE days 13–17; P = 0.05). Interestingly, Cp knockout mice also exhibited a significant increase in the number of astrocytes with reactive morphology in early EAE compared with wild‐type mice at the same stage of disease. CNS iron levels were not increased with EAE in these mice. Based on these observations, we propose that an increase in Cp expression could contribute to tissue damage in early EAE. In addition, endogenous CP either directly or indirectly inhibits astrocyte reactivity during early disease, which could also worsen early disease evolution. © 2014 Wiley Periodicals, Inc.     

Biochemical testing for the diagnosis of Wilson's disease: A systematic review

BackgroundWilson''s disease (WD) is a rare inherited disorder that leads to copper accumulation in the liver, brain, and other organs. WD is prevalent worldwide, with an occurrence of 1 per 30,000 live births. Currently, there is no gold standard diagnostic test for WD. The objective of this systematic review is to determine the diagnostic accuracy for WD of three biochemical tests, namely hepatic copper, 24‐hour urinary copper, and ceruloplasmin using the Leipzig criteria.MethodsAdhering to PRISMA guidelines, databases including PubMed/MEDLINE, CINAHL Plus, Web of Science, and Cochrane were searched. Studies that comprised of confirmed or suspected WD along with normal populations were included with adult and pediatric group. The sensitivity, specificity, negative predictive value and positive predictive value were computed using RevMan 5.4.ResultsNine studies were included. The best practice evidence for 24‐hour urinary copper test ranged from a cutoff value of 0.64–1.6 μmol/24 h (N = 268; sensitivity = 75.6%, specificity = 98.3%). Hepatic copper test was optimally cutoff based on the ROC curve analysis at 1.2 μmol/g yielding a power of 96.4% sensitivity and 95.4% specificity (N = 1,150); however, the tried and tested 4 μmol/g cutoff, with 99.4% sensitivity and 96.1% specificity, is more widely accepted. The ceruloplasmin test cutoff value was found to be ranging from 0.14 to 0.2 g/L (N = 4,281; sensitivity = 77.1%–99%, specificity = 55.9%–82.8%).ConclusionThis paper provides a large‐scale analysis of current evidence pertaining to the biochemical diagnosis of WD employing the Leipzig criteria. The laboratory values are typically based on specific subgroups based on age, ethnicity, and clinical subgroups. The findings of this systematic review must be used with caution, given the over‐ or under‐estimation of the index tests.     

Direct Measurement of ATP7B Peptides Is Highly Effective in the Diagnosis of Wilson Disease

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