Reference SummarySun X, Mol Cancer 2009;8():73

Title

The proprotein convertase PC5/6 is protective against intestinal tumorigenesis: in vivo mouse model.

Authors

Sun X; Essalmani R; Seidah NG; Prat A

Journal

Mol Cancer

Volume

8

Issue

Year

2009

Pages

73

Abstract

BACKGROUND: The secretory basic amino acid-specific proprotein convertases (PCs) have often been associated with cancer/metastasis. By controlling the cleavage of cancer-associated proteins, PCs play key roles in multiple steps of cancer development. Most analyses of the implication of PCs in cancer/metastasis relied on the use of in vitro overexpression systems or inhibitors that can affect more than one PC. Aside from the role of furin in salivary gland tumorigenesis, no other in vivo genetic model of PC-knockout was reported in relation to cancer development. RESULTS: Since PC5/6 is highly expressed in the small intestine, the present study examined its in vivo role in intestinal tumorigenesis. Analysis of human intestinal tumors at various stages showed a systematic down-regulation of PC5/6 expression. Since gene inactivation of PC5/6 leads to lethality at birth, we generated mice lacking PC5/6 in enterocytes and analyzed the impact of the presence or absence of this PC in the mouse ApcMin/+ model that develops numerous adenocarcinomas along the intestinal tract. This resulted in viable mice with almost no expression of PC5/6 in small intestine, but with no overt phenotype. The data showed that by themselves ApcMin/+ tumors express lower levels of PC5/6 mRNA, and that the lack of PC5/6 in enterocytes results in a significantly higher tumor number in the duodenum, with a similar trend in other intestinal segments. Finally, the absence of PC5/6 is also associated with a premature mortality of ApcMin/+ mice. CONCLUSION: Overall, these data suggest that intestinal PC5/6 is protective towards tumorigenesis, especially in mouse duodenum, and possibly in human colon.

Links

J:220950 – MGI References
19737405 – National Library of Medicine/PubMed

Models

Strain Model Name Treatment Agent(s) Organ Affected Frequency Model Details
STOCK ApcMin/+ Pcsk5tm2Prat Intestine - Large Intestine - Colon tumor Intestine - Large Intestine - Colon

63.64

STOCK ApcMin/+ Pcsk5tm2Prat Tg(Vil1-cre)997Gum Intestine - Large Intestine - Colon tumor Intestine - Large Intestine - Colon

52.94

C57BL/6J-ApcMin/+ Intestine - Small Intestine - Duodenum tumor Intestine - Small Intestine - Duodenum

observed

STOCK ApcMin/+ Pcsk5tm2Prat Intestine - Small Intestine - Duodenum tumor Intestine - Small Intestine - Duodenum

90.91

STOCK ApcMin/+ Pcsk5tm2Prat Tg(Vil1-cre)997Gum Intestine - Small Intestine - Duodenum tumor Intestine - Small Intestine - Duodenum

100

C57BL/6J-ApcMin/+ Intestine - Small Intestine - Ileum tumor Intestine - Small Intestine - Ileum

observed

STOCK ApcMin/+ Pcsk5tm2Prat Intestine - Small Intestine - Ileum tumor Intestine - Small Intestine - Ileum

100

STOCK ApcMin/+ Pcsk5tm2Prat Tg(Vil1-cre)997Gum Intestine - Small Intestine - Ileum tumor Intestine - Small Intestine - Ileum

100

C57BL/6J-ApcMin/+ Intestine - Small Intestine - Jejunum tumor Intestine - Small Intestine - Jejunum

observed

STOCK ApcMin/+ Pcsk5tm2Prat Intestine - Small Intestine - Jejunum tumor Intestine - Small Intestine - Jejunum

100

STOCK ApcMin/+ Pcsk5tm2Prat Tg(Vil1-cre)997Gum Intestine - Small Intestine - Jejunum tumor Intestine - Small Intestine - Jejunum

100

C57BL/6J-ApcMin/+ Intestine - Small Intestine tumor Intestine - Small Intestine

observed

C57BL/6J-ApcMin/+ Intestine adenocarcinoma Intestine

observed

STOCK ApcMin/+ Pcsk5tm2Prat Intestine tumor Intestine

100

STOCK ApcMin/+ Pcsk5tm2Prat Tg(Vil1-cre)997Gum Intestine tumor Intestine

100