简介：全身的豺狼座erythematosus(SLE)的一个特点是由汽车反应的B房间的各种各样的自身抗体的一致生产。Interferon-α；(IFN-α；)发信号高度在SLEB房间被激活并且由B房间在抗体反应起一个重要作用。以前的研究证明了CD180否定的B房间，戏剧性地在SLE病人被增加，为自身抗体的生产负责。然而，在CD180和IFN-α之间的协会；未知的发信号的遗体。在现在的学习，我们在调整IFN-α的激活上探索了CD180的效果；在B房间发信号。我们发现CD180否定的B房间的数字在MRL/Mp-Fas(lpr/lpr)被增加豺狼座容易的老鼠与野类型的老鼠相比。Phenotypic分析证明CD180否定的B房间包括了CD138+plasmablast/plasma房间和GL-7+幼芽的中心(GC)B房间。尤其是，CD180的结扎显著地禁止了IFN-α；信号变换器的导致的phosphorylation和抄写2的使活跃之物(STAT-2)和以在vitro的一种Lyn-PI3K-BTK-dependent方式的刺激IFN的基因(ISG)的表示。而且，CD180的结扎能也禁止IFN-α；在在vivo的B房间的导致的ISG表示。而且，像使用费的受体7并且像使用费的受体9发信号小径能显著地downregulateCD180表示并且调制在IFN-α的激活上发信号的CD180的禁止的效果；发信号。一起，我们的结果加亮在CD180否定的B房间的增加的比例和IFN-α的激活之间的靠近的协会；在SLE发信号。我们的数据提供分子的卓见进IFN-α的机制；在为SLE处理的SLEB房间和一条潜在的治疗学的途径的发信号的激活。

简介：AbstractBackground:Patients carrying the HongKongαα (HKαα) allele and -α3.7/αααanti-4.2 could be misdiagnosed as -α3.7/αα by the current conventional thalassemia detection methods, leading to inaccurate genetic counseling and an incorrect prenatal diagnosis. This study was aimed to accurately analyze the genotypes of HKαα carriers and -α3.7/αααanti-4.2.Methods:Samples were collected in our hospital from July 2017 to October 2019. Twenty-four common types of Chinese thalassemia were screened by gap-polymerase chain reaction (Gap-PCR) and reverse dot blot (RDB). Anti-4.2 multiplex-PCR was used to confirm carriers of the αααanti-4.2 duplication with -α3.7 deletion. Two-round nested PCR and multiplex ligation-dependent probe amplification (MLPA) were applied to accurately identify and confirm their genotypes. For data analysis, we used descriptive statistics and Fisher’s exact tests.Results:Two thousand five hundred and forty-four cases were identified as thalassemia in 5488 peripheral blood samples. The results showed that α, β, and αβ compound thalassemia were identified in 1190 (46.78%), 1286 (50.55%), and 68 (2.67%) cases, respectively. A total of 227 samples from thalassemia patients were identified as -α3.7/αα by Gap-PCR, and the genotypes of two samples were uncertain. There was a difference between Gap-PCR and combined groups (Gap-PCR combined with nested PCR and MLPA) in detecting HKαα (P < 0.05). Among the 229 patients, 20 patients were identified as HKαα carriers and one was identified as -α3.7/αααanti-4.2 by two-round nested PCR and MLPA, including 15 patients with HKαα/αα, three with HKαα/αα and β-thalassemia coinheritance, one with HKαα/-SEA, one with HKαα/-α4.2 and β-thalassemia coinheritance, and one with -α3.7/αααanti-4.2 and β-thalassemia coinheritance.Conclusions:αααanti-4.2 and HKαα genotypes of patients carrying -α3.7 need to be detected to reduce the misdiagnosis rate of patients carrying HKαα and -α3.7/αααanti-4.2 alleles. More accurate genetic counseling can be provided in the clinic using nested PCR combined with MLPA.

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