P3H2 Q8IVL5

脯氨酰3-羟化酶2 (EC 1.14.11.7) (Leprecan样蛋白1) (黏液样脂肪肉瘤相关蛋白4)

功能描述

脯氨酰3-羟化酶，催化胶原蛋白上3-羟脯氨酸的翻译后形成 (PubMed:18487197)。参与肌腱、眼巩膜和眼晶状体囊中胶原蛋白COL4A1和COL1A1的脯氨酸3-羟化 (By similarity)。对IV型胶原蛋白COL4A1具有高活性，对COL1A1活性较低 (PubMed:18487197)。催化Gly-Pro-Hyp序列中第一个Pro的羟化，其中Hyp为4-羟脯氨酸 (PubMed:18487197)。对第三位缺乏4-羟脯氨酸的底物没有活性 (PubMed:18487197)。 {ECO:0000250|UniProtKB:Q8CG71, ECO:0000269|PubMed:18487197}。

组织特异性

Expression localized to the epithelia of bile ducts and to the sacroplasm of heart muscle and skeletal muscle. In the pancreas, localized to a subpopulation of Langerhans islet cells and in the salivary gland, expressed in acinar cells (at protein level) (PubMed:15063763). Expressed in adult heart, placenta, lung, liver, skeletal muscle and kidney (PubMed:15063763, PubMed:18487197). Detected in fetal heart, spleen, lung, liver skeletal muscle and kidney (PubMed:18487197).

亚细胞定位

Endoplasmic reticulum