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Neurology. 1994 Jun;44(6):1083-6.

Turcot's syndrome: evidence for linkage to the adenomatous polyposis coli (APC) locus.

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1
Department of Neurology, College of Physicians and Surgeons of Columbia University, New York, NY.

Abstract

OBJECTIVE:

To investigate the possibility that neuroepithelial tumors in Turcot's syndrome are caused by pleiotropic mutations in the gene for adenomatous polyposis coli (APC), a tumor-suppressor gene implicated in colonic cancer.

METHODS:

We studied the inheritance patterns of genetic markers for the chromosome 5q21 region in 12 members of a Turcot's syndrome kindred with five affected members. We performed linkage analysis to detect linkage between the disease phenotype and DNA markers.

RESULTS:

Marker D5S346, located 30 to 70 kilobases from the APC locus, showed evidence highly suggestive of linkage to the disease phenotype (lod score = 1.92).

CONCLUSIONS:

The data provide evidence that the tumor-suppressor gene implicated in APC and sporadic colon cancers may also cause malignant neuroepithelial tumors in Turcot's syndrome.

PMID:
8208405
[Indexed for MEDLINE]
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