Disruption of a Novel Imprinted Zinc-finger Gene, ZNF215, in Beckwith-Wiedemann Syndrome

Overview

Journal Am J Hum Genet

Publisher Cell Press

Specialty Genetics

Date 2000 Apr 14

PMID 10762538

Citations 19

Authors

M Alders

A Ryan

M Hodges

J Bliek

A P Feinberg

O Privitera

A Westerveld

P F LITTLE

M Mannens

Affiliations

Soon will be listed here.

Abstract

The genetics of Beckwith-Wiedemann syndrome (BWS) is complex and is thought to involve multiple genes. It is known that three regions on chromosome 11p15 (BWSCR1, BWSCR2, and BWSCR3) may play a role in the development of BWS. BWSCR2 is defined by two BWS breakpoints. Here we describe the cloning and sequence analysis of 73 kb containing BWSCR2. Within this region, we detected a novel zinc-finger gene, ZNF215. We show that two of its five alternatively spliced transcripts are disrupted by both BWSCR2 breakpoints. Parts of the 3' end of these splice forms are transcribed from the antisense strand of a second zinc-finger gene, ZNF214. We show that ZNF215 is imprinted in a tissue-specific manner.

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