蛋白前体的γ-羧基化
中文名称
通路描述
凝血酶、因子 VII、因子 IX、因子 X、蛋白 C、蛋白 S、蛋白 Z 和 Gas 6 等蛋白的氨基末端附近的一组谷氨酸残基的γ-羧基化对于这些蛋白结合 Ca++并高效参与血液凝固至关重要。一种单一的维生素 K 依赖性γ-羧基化酶催化所有 8 种参与凝血（Morris et al. 1995; Brenner et al. 1998; Spronk et al. 2000）的蛋白的γ-羧基化。在羧基化反应中，酶通过位于要羧基化的谷氨酸残基侧链氨基末端序列基序结合其底物蛋白，然后依次羧基化簇中的所有谷氨酸，释放底物（Morris et al. 1995; Berkner 2000; Stenina et al. 2001）。该反应发生在内质网（Bristol et al. 1996）。
英文描述
Gamma-carboxylation of protein precursors Gamma-carboxylation of a cluster of glutamate residues near the amino termini of thrombin, factor VII, factor IX, factor X, protein C, protein S, protein Z, and Gas 6 is required for these proteins to bind Ca++ and function efficiently in blood clotting. A single enzyme, vitamin K-dependent gamma-carboxylase, catalyzes the gamma-carboxylation of all eight proteins involved in clotting (Morris et al. 1995; Brenner et al. 1998; Spronk et al. 2000). In the carboxylation reaction, the enzyme binds its substrate protein via a sequence motif on the amino terminal side of the glutamate residues to be carboxylated (Furie et al. 1999), then processively carboxylates all glutamates in the cluster before releasing the substrate (Morris et al. 1995; Berkner 2000; Stenina et al. 2001). The reaction occurs in the endoplasmic reticulum (Bristol et al. 1996).
所含基因
10 个基因