Purification and characterization of an abnormal factor IX (Christmas factor) molecule. Factor IX Chapel Hill.
Purification and characterization of an abnormal factor IX (Christmas factor) molecule. Factor IX Chapel Hill.
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异常因子 IX（圣诞因子）分子的纯化和表征。
DOI：
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发表时间：
1978
影响因子：
15.9
通讯作者：
H. Roberts
中科院分区：
文献类型：
--
作者：
K. Chung;D. Madar;J. Goldsmith;H. Kingdon;H. Roberts
从轻度血友病B患者的血浆中分离出人因子IX（Christmas因子）。患者的血浆含有5%的因子IX凝血活性，但100%的因子IX抗原活性，如通过免疫学测定所确定的，所述免疫学测定包括抑制剂中和和因子IX的放射免疫测定。这种异常的因子IX被称为因子IX查佩尔山（Factor IXCH）。正常的因子IX和因子IXCH都具有酪氨酸作为NH 2末端氨基酸。这两种蛋白质具有相似的分子量、相似的氨基酸分析、相同数量的γ-羧基谷氨酸残基（10个γ-羧基谷氨酸残基）和相似的碳水化合物含量。两者都以单链糖蛋白的形式存在于血浆中。正常因子IX和因子IXCH之间的主要区别在于，后者在因子XIa和Ca 2+存在下表现出对因子IXa的延迟活化。因此，因子IXCH不同于其他先前描述的异常因子IX分子。
Human Factor IX (Christmas factor) was isolated from the plasma of a patient with mild hemophilia B. The patient's plasma contained 5% Factor IX clotting activity but 100% Factor IX antigenic activity as determined by immunological assays, which included inhibitor neutralization and a radioimmunoassay for Factor IX. This abnormal Factor IX is called Factor IX Chapel Hill (Factor IXCH). Both normal Factor IX and Factor IXCH have tyrosine as the NH2-terminal amino acid. The two proteins have a similar molecular weight, a similar amino acid analysis, the same number of gamma-carboxyglutamic acid residues (10 gamma-carboxyglutamic acid residues), and a similar carbohydrate content. Both exist as a single-chain glycoprotein in plasma. The major difference between normal Factor IX and Factor IXCH is that the latter exhibits delayed activation to Factor IXa in the presence of Factor XIa and Ca2+. Thus, Factor IXCH differs from other previously described abnormal Factor IX molecules.