P1PK、GLOB和FORS血型系统以及GLOB血型集合：生化与临床方面。我们是否已经完全理解了呢？
P1PK, GLOB, and FORS blood group systems and GLOB collection: biochemical and clinical aspects. Do we understand it all yet?
作者信息
Kaczmarek Radoslaw, Buczkowska Anna, Mikołajewicz Katarzyna, Krotkiewski Hubert, Czerwinski Marcin
机构信息
Laboratory of Glycoconjugate Immunochemistry, Ludwik Hirszfeld Institute of Immunology and Experimental Therapy, Wrocław, Poland.
Laboratory of Glycoconjugate Immunochemistry, Ludwik Hirszfeld Institute of Immunology and Experimental Therapy, Wrocław, Poland; Institute of Biochemistry and Biophysics, Warsaw, Poland.
出版信息
Transfus Med Rev. 2014 Jul;28(3):126-36. doi: 10.1016/j.tmrv.2014.04.007. Epub 2014 Apr 27.
Antigens belonging to the P1PK, GLOB, and FORS blood group systems and the GLOB blood group collection represent a closely related set of 13 glycosphingolipids (GSLs). They are synthesized by the coordinated action of glycosyltransferases, encoded by at least 7 different loci. Three of these enzymes show either different activity or a different mRNA expression profile due to genetic polymorphisms, resulting in blood group diversity. In recent years, significant progress has been made in understanding the molecular background and biological functions of these GSLs. Their medical significance is often related to the existence of natural antibodies, as they may cause complications after transfusions and during pregnancies. In addition, GSLs belonging to these blood group systems are receptors for several pathogens. This review summarizes the present knowledge about the complicated network of enzymatic interactions leading to synthesis of these GSLs, as well as their clinical implications.
属于P1PK、GLOB和FORS血型系统以及GLOB血型集合的抗原代表了一组密切相关的13种糖鞘脂（GSL）。它们是由糖基转移酶协同作用合成的，这些糖基转移酶由至少7个不同的基因座编码。由于基因多态性，其中三种酶表现出不同的活性或不同的mRNA表达谱，从而导致血型多样性。近年来，在理解这些GSL的分子背景和生物学功能方面取得了重大进展。它们的医学意义通常与天然抗体的存在有关，因为它们可能在输血后和怀孕期间引起并发症。此外，属于这些血型系统的GSL是几种病原体的受体。本综述总结了目前关于导致这些GSL合成的复杂酶促相互作用网络的知识及其临床意义。
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