Reference SummarySmits R, Gastroenterology 1998 Feb;114(2):275-83

Title

Apc1638N: a mouse model for familial adenomatous polyposis-associated desmoid tumors and cutaneous cysts.

Authors

Smits R; van der Houven van Oordt W; Luz A; Zurcher C; Jagmohan-Changur S ; Breukel C ; Khan PM ; Fodde R

Journal

Gastroenterology

Volume

114

Issue

2

Year

1998

Pages

275-83

Abstract

Background & Aims: Germline mutations in the adenomatous polyposis coli (APC) gene are responsible for familiar adenomatous polyposis (FAP), an autosomal dominant predisposition to the formation of multiple colorectal adenomas. Moreover, patients with FAP are at high risk of developing several extracolonic manifestations, including desmoids, cutaneous cysts, and tumors of the upper gastrointestinal tract. Although by definition desmoids are nonmalignant, because of their aggressive invasion of local structures, they represent one of the major causes of morbidity and mortality among patients with FAP. Methods:This study describes the histopathologic and molecular characterization of Apc1638N, a mouse model for the broad spectrum of extracolonic manifestations characteristic of FAP. Results: Heterozygous Apc(+)/ Apc1638N animals develop fully penetrant and multifocal cutaneous follicular cysts and desmoid tumors in addition to attenuated polyposis of the upper gastrointestinal tract. Moreover, breeding of Apc(+)/Apc1638N mice in a p53- deficient background results in a dramatic sevenfold increase of the desmoid multiplicity. Conclusions: Because of the attenuated nature of their intestinal phenotype, these mice survive longer than other murine models for Ape- driven tumorigenesis. Therefore, Apc1638N represents an ideal laboratory tool to test various therapeutic intervention strategies for the management of intestinal as well as extraintestinal tumors.

Links

J:46025 – Mouse Genome Informatics
9453487 – National Library of Medicine/PubMed

Models

Strain Model Name Treatment Agent(s) Organ Affected Frequency Model Details
B6.129-Apctm1Rak/+ Connective tissue - Fibroblast desmoid Muscle - Striated

100

B6.129-Apctm1Rak/+ Connective tissue - Fibroblast desmoid Peritoneum - Mesentery

100

C57BL/6J-ApcMin/+ Connective tissue - Fibroblast desmoid Skin

15

B6.129-Apctm1Rak/+ Connective tissue - Fibroblast desmoid Muscle - Striated - Skeletal

100

B6.129-Apctm1Rak/+ Trp53tm1Tyj/+ Connective tissue - Fibroblast desmoid Muscle - Striated - Skeletal

100

B6.129-Apctm1Rak/+ Trp53tm1Tyj Connective tissue - Fibroblast desmoid Muscle - Striated - Skeletal

100

B6.129-Apctm1Rak/+ Skin gland - Sebaceous gland cyst Skin gland - Sebaceous gland

50 - 100

C57BL/6J-ApcMin/+ Skin gland - Sebaceous gland cyst Skin gland - Sebaceous gland

92