Jackson Laboratory

A/J Physiological Data

A strain characteristic of A/J (000646) is a late onset (four to five months) progressive muscular dystrophy as a result of a homozygous retrotransposon insertion in the dysferlin (Dysf) gene. Myofibers in Dysf^prmd homozygotes undergo degeneration and regeneration, and their nuclei are placed centrally. Proximal muscles are more severely affected than distal muscles. These mice model dysferlin deficiency, an adult-onset muscular dystrophy.

Return to Muscular Dystrophy Efficacy Studies

Morphometry

Automated Morphometry Measurements

Fiber Areas in the Psoas

Automated morphometry measurements plot the frequency distribution of muscle fibers based on their diameter and detects atrophy (increase in small fibers). A/J mice have an increase in atrophy compared to wildtype (WT), further potentiated by a test article.