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.

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