University of Iowa Research Foundation

Madeleine Durbeej

Kevin P Campbell

Roger Williamson

Ronald Cohn

Ramon Coral

Disclosed is a mouse, cells derived therefrom, and methods for using the mouse, the mouse being homozygous for a disrupted &dgr;-sarcoglycan gene, the disruption in the gene having been introduced into the mouse or an ancestor of the mouse at an embryonic stage. The disruption prevents the synthesis of functional &dgr;-sarcoglycan in cells of the mouse and results in the mouse having a reduced amount of &bgr;- and &egr;-sarcoglycan and sarcospan, and a disruption of the sarcoglycan-sarcospan complex in smooth muscle of the mouse. Also disclosed is a mouse, cells derived therefrom, and methods for using the mouse, the mouse being homozygous for a disrupted &bgr;-sarcoglycan gene, the disruption in the gene having been introduced into the mouse or an ancestor of the mouse at an embryonic stage. The disruption prevents the synthesis of functional &bgr;-sarcoglycan in cells of the mouse and results in the mouse having a reduced amount of &dgr;-and &egr;-sarcoglycan and sarcospan and &agr;-dystroglycan in smooth muscle of the mouse.

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Iowa City, IA, United States of America

Madeleine Durbeej