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La/SS-B

La/SS-B

产品货号:12800/12801
促销: 0.00
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0.1mg 1mg
德国Diarect

Diseases:
Systemic Lupus Erythematosus (SLE)
Sjøgren Syndrome, Subacute Cutaneous LE
 

The La/SS-B antigen is a 50-kDa nuclear phosphoprotein which – based on sequence homology analysis – appears to be composed of an ATP binding and an RNA binding domain. Several cellular functions of the La/SS-B protein, mostly involved with RNA metabolism, are being discussed, of which an involvement in the correct and efficient termination of RNA polymerase III transcripts has been proven.

La/SS-B antibodies occur with a prevalence of 40-90% in Sjøgren syndrome patients but also with a prevalence of 5-15% in SLE patients. Similar to the Ro/SS-A situation high prevalences of La/SS-B antibodies also occur in subacute cutaneous LE and neonatal lupus syndrome where a high risk of neonatal lupus exists if the mother is positive for Ro/SS-A (52 kDa) and La/SS-B antibodies.

La/SS-B protein from DIARECT is expressed in the baculovirus expression system.

 

 

相关疾病: 系统性红斑狼疮(SLE),干燥综合征,亚急性皮肤型红斑狼疮(SCLE)干燥综合症是一种自身免疫紊乱疾病,以淋巴细胞浸润和破坏唾液腺、泪腺为特征。该疾病的血清学特征是系统产生一种核蛋白微粒的自身抗体,包含蛋白Ro/SS-A(60kDa和52kDa),La/SS-B(48kDa)和小的细胞浆RNAs(hY RNAs)。这一疾病单独发生时被认为是原发性的干燥综合症,当它和其他的结缔组织病如系统性红斑狼疮(SLE)同时发生就被认为是继发性的干燥综合症,抗Ro和抗La抗体在患有SLE和干燥综合症的绝大多数的患者中都存在。在狼疮患者中,这些抗体和光敏性皮肤损害高度相关,特别是在亚急性皮肤红斑狼疮(SCLE)和新生儿红斑狼疮(Neonatal LE)的患者中。
    La/SS-B抗原是一个分子量为50kDa的核磷蛋白,基于序列同源性分析,发现其各有一个ATP结合位点和RNA结合位点。La/SS-B蛋白的细胞内功能有参与RNA代谢和RNA聚合酶III转录的准确有效终止。40-90%的干燥综合症患者有La/SS-B抗体, 5-15%的系统性红斑狼疮患者也有该抗体,与Ro/SS-A相似,在亚急性皮肤型红斑狼疮患者和新生儿红斑狼疮患者血清中存在抗La/SS-B抗体的比例也很高。如果母亲检出抗Ro/SS-A (52 kDa)抗体and抗La/SS-B抗体阳性,则新生儿患新生儿红斑狼疮的风险很高。

DIARECT生产的重组人La/SS-B蛋白是在杆状病毒表达系统中表达的。