Motor End-Plate Disease, Mouse

Abstract

Affected mice are much smaller than unaffected littermates. Most affected mice die at 18–25 days. At this age, they typically weigh 5 g compared with unaffected mice, at 8 g. Because the gene for med is linked to the gene for caracul, affected mice have a smooth, sleek coat whereas unaffected mice have a curled coat. Affected mice are weak, do not move freely, fall frequently, and exhibit profound muscle wasting, especially in the forelimbs and in proximal muscles of both pectoral and pelvic girdles and the trunk and face (Duchen and Searle 1970).