Pajunen L, Jones T A, Goddard A, Sheer D, Solomon E, Pihlajaniemi T, Kivirikko K I
Department of Medical Biochemistry, University of Oulu, Finland.
Cytogenet Cell Genet. 1991;56(3-4):165-8. doi: 10.1159/000133078.
Prolyl 4-hydroxylase, an alpha 2 beta 2 tetramer, catalyzes the formation of 4-hydroxyproline in collagens and related proteins by hydroxylating proline residues in peptide linkages. The beta-subunit of prolyl 4-hydroxylase (P4HB) is a highly unusual multifunctional polypeptide that is identical to the enzyme protein disulfide isomerase and a major cellular thyroid hormone-binding protein and is highly similar to a glycosylation site-binding polypeptide of oligosaccharyl transferase. We report here the regional assignment of the gene for this multifunctional polypeptide. In situ hybridization mapped the gene to 17q25. Southern blot analyses of restricted DNA from a chromosome-mediated gene transfer transfectant panel suggested that the P4HB gene is located distal to the gene for thymidine kinase, either between the genes for thymidine kinase and galactokinase or on the telomeric side of both these genes.
脯氨酰4-羟化酶是一种α2β2四聚体，通过羟基化肽键中的脯氨酸残基来催化胶原蛋白及相关蛋白质中4-羟脯氨酸的形成。脯氨酰4-羟化酶（P4HB）的β亚基是一种非常特殊的多功能多肽，它与酶蛋白二硫键异构酶相同且是一种主要的细胞甲状腺激素结合蛋白，还与寡糖基转移酶的糖基化位点结合多肽高度相似。我们在此报告该多功能多肽基因的区域定位。原位杂交将该基因定位于17q25。对染色体介导的基因转移转染细胞系的限制性DNA进行的Southern印迹分析表明，P4HB基因位于胸苷激酶基因的远端，要么在胸苷激酶基因和半乳糖激酶基因之间，要么在这两个基因的端粒侧。
