Structural organization and expression patterns of the human and mouse genes for the type I procollagen COOH-terminal proteinase enhancer protein

The procollagen C-proteinase enhancer (PCPE) is a glycoprotein that potenti ates enzymatic cleavage of the type I procollagen C-propeptide by bone morp hogenetic protein-1 (BMP-1). The human PCPE gene (PCOLCE) was previously ma pped to 7q22, an area frequently disrupted in uterine leiomyomata, while di sruption of the rat PCPE gene leads to anchorage-independent growth and los s of contact inhibition in rat fibroblasts. Here we describe the entire int ron/ exon organizations of PCOLCE and the mouse PCPE gene (Pcolce) and anal yze expression of PCOLCE RNA in various human adult and fetal tissues and o f Pcolce RNA at various stages of mouse development. PCOLCE and Pcolce are shown to be small genes 6.0 and 6.5 kb, respectively, with a conserved intr on/exon structure comprising 9 exons. A notable difference between the two genes derives from insertion of multiple Alu sequences immediately upstream and downstream and within PCOLCE. Temporal expression of PCPE mRNA is show n to differ from that of BMP-1 and type I procollagen during mouse developm ent, consistent with possible additional functions for PCPE beyond enhancem ent of C-proteinase activity. Consistent with a possible role in leiomyomat a, PCOLCE is shown to be expressed at relatively high levels in uterus. (C) 1999 Academic Press.