MOLECULAR CHARACTERIZATION OF SIALOPHORIN (CD43), THE LYMPHOCYTE SURFACE SIALOGLYCOPROTEIN DEFECTIVE IN WISKOTT-ALDRICH SYNDROME
MOLECULAR CHARACTERIZATION OF SIALOPHORIN (CD43), THE LYMPHOCYTE SURFACE SIALOGLYCOPROTEIN DEFECTIVE IN WISKOTT-ALDRICH SYNDROME
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DOI：
10.1073/pnas.86.8.2819
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发表时间：
1989-04-01
影响因子：
11.1
通讯作者：
WHITEHEAD, AS
中科院分区：
文献类型：
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作者：
SHELLEY, CS;REMOLDODONNELL, E;WHITEHEAD, AS
白细胞和血小板的唾液酸蛋白（CD 43）是一种表面唾液酸糖蛋白，在X染色体连锁免疫缺陷Wiskott-Aldrich综合征患者的淋巴细胞上是表型缺陷的。以前的单克隆抗体研究表明，唾液酸蛋白是T淋巴细胞活化途径的一个组成部分。在这里，我们描述的cDNA克隆和衍生的氨基酸序列的人唾液酸蛋白。该序列预测了一个完整的膜多肽与N-末端疏水信号区，随后是一个粘蛋白样的235个残基的胞外区，均匀分布的46个丝氨酸，47个苏氨酸和24个脯氨酸残基。随后是23个残基的跨膜区和123个残基的C-末端胞内区。这些区域在进化过程中高度保守;细胞内区域含有许多可能介导活化信号转导的潜在磷酸化位点。唾液酸蛋白基因的染色体定位进行了测定和Wiskott-Aldrich综合征的发病机制，这一任务的影响进行了讨论。
Sialophorin (CD43) of leukocytes and platelets is a surface sialoglycoprotein that is phenotypically defective on lymphocytes of patients with the X chromosome-linked immunodeficiency Wiskott-Aldrich syndrome. Previous studies with monoclonal antibodies indicate that sialophorin is a component of a T-lymphocyte activation pathway. Here we describe the cDNA cloning and derived amino acid sequence of human sialophorin. The sequence predicts an integral membrane polypeptide with an N-terminal hydrophobic signal region followed by a mucin-like 235-residue extracellular region with a uniform distribution of 46 serine, 47 threonine, and 24 proline residues. This is followed by a 23-residue transmembrane region and a 123-residue C-terminal intracellular region. These latter regions have been highly conserved during evolution; the intracellular region contains a number of potential phosphorylation sites that might mediate transduction of activation signals. The chromosomal location of the sialophorin gene was determined and the implications of this assignment for the pathogenesis of the Wiskott-Aldrich syndrome are discussed.