人 KEL 抗体对
01AB00864
Kell metallo-endopeptidase (KEL), also known as the Kell blood group glycoprotein, is a type II transmembrane zinc endopeptidase primarily recognized for its role in the Kell blood group system on red blood cells. Structurally, it is a 93-kDa glycoprotein with two globular domains, containing an active site that coordinates zinc and catalyzes the conversion of big endothelin-3 to its active, vasoconstrictive form, with a marked preference for endothelin-3 over other substrates. The Kell protein is linked to the XK protein via a disulfide bond, and both are expressed mainly in erythroid tissues but also in other tissues. KEL is highly polymorphic, with over 20 antigens resulting from single amino acid substitutions, making it significant in transfusion medicine due to the immunogenicity of certain variants. Clinically, antibodies against Kell antigens, particularly KEL1, can cause severe transfusion reactions and suppress erythropoiesis, leading to fetal anemia in sensitized pregnancies; absence of XK (McLeod phenotype) is associated with acanthocytosis and neuromuscular symptoms.
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技术参数
- 靶点基因名
- KEL
- 蛋白别名
- Kell metallo-endopeptidase (Kell blood group); KEL
- UniProt
- P23276
- 物种
- Human
产品详情
- 靶点名称
- KEL
- 蛋白名称
- Kell metallo-endopeptidase (Kell blood group)
- 蛋白别名
- Kell blood group glycoprotein; CD antigen CD238
- 靶点基因名
- KEL
- UniProt
- P23276
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Kell metallo-endopeptidase (KEL), also known as the Kell blood group glycoprotein, is a type II transmembrane zinc endopeptidase primarily recognized for its role in the Kell blood group system on red blood cells. Structurally, it is a 93-kDa glycoprotein with two globular domains, containing an active site that coordinates zinc and catalyzes the conversion of big endothelin-3 to its active, vasoconstrictive form, with a marked preference for endothelin-3 over other substrates. The Kell protein is linked to the XK protein via a disulfide bond, and both are expressed mainly in erythroid tissues but also in other tissues. KEL is highly polymorphic, with over 20 antigens resulting from single amino acid substitutions, making it significant in transfusion medicine due to the immunogenicity of certain variants. Clinically, antibodies against Kell antigens, particularly KEL1, can cause severe transfusion reactions and suppress erythropoiesis, leading to fetal anemia in sensitized pregnancies; absence of XK (McLeod phenotype) is associated with acanthocytosis and neuromuscular symptoms.