Cloning and characterization of the cDNA coding for a polymyositis-scleroderma overlap syndrome-related nucleolar 100-kD protein.

Cloning and characterization of the cDNA coding for a polymyositis-scleroderma overlap syndrome-related nucleolar 100-kD protein.
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DOI:
10.1084/jem.176.4.973
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发表时间:
1992-10-01
期刊:
The Journal of experimental medicine
影响因子:
--
通讯作者:
Bautz FA
Bautz FA
中科院分区:
其他
文献类型:
--
作者:
Blüthner M;Bautz FA

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据报道,约50%的多发性肌炎-硬皮病重叠综合征患者具有对称为PM/Scl的核仁颗粒的自身抗体。该颗粒由几种多肽组成,其中75和100 kD的两种蛋白质已被鉴定为主要抗原组分。在这里,我们报告的cDNA克隆和部分表位定位的100 kD的自身抗原从人胎盘和HeLa λ gt 11库。推导的氨基酸序列编码885个氨基酸残基的蛋白质,分子量为100.8 kD。针对重组蛋白片段的兔抗体以与针对核仁100 kD蛋白的人自身抗体相同的方式在免疫荧光和免疫印迹中反应。序列分析表明,从丝氨酸/苏氨酸激酶的12个氨基酸的一致序列的同源性密切,这表明这种自身抗原的可能功能。发现主要抗原区域位于多肽的NH 2-末端三分之一内。
About 50% of patients with the polymyositis-scleroderma overlap syndrome are reported to have autoantibodies to a nucleolar particle termed PM/Scl. The particle consists of several polypeptides of which two proteins of 75 and 100 kD have been identified as the major antigenic components. Here we report on the cDNA cloning and partial epitope mapping of the 100-kD autoantigen from human placenta and HeLa lambda gt11 libraries. The deduced amino acid sequence encodes a protein of 885 amino acid residues with a molecular mass of 100.8 kD. Rabbit antibodies raised against a recombinant protein fragment reacted in immunofluorescence and immunoblotting in the same manner as human autoantibodies directed against the nucleolar 100-kD protein. Sequence analysis shows close homology to a consensus sequence of 12 amino acids from serine/threonine kinases, suggesting a possible function for this autoantigen. A major antigenic region is found to be located within the NH2-terminal third of the polypeptide.