Summary
Congenic mice of the MRL +/+ substrain provide an animal model for study of tubular aggregates in skeletal muscle. Tubular aggregates appear limited only to males of the MRL +/+ strain and are not found in other strains, including MRL +/−, MRL lpr/lpr, BXSB/MpJ, BALB/c, SJL/J, AJ, or C3H HEJ. This strain-specific occurrence, and the gradually increasing abundance of tubular aggregates after 6 months of age until virtually all type II myofibers are affected by 2 years, implies that both genetic and developmental factors are necessary for the occurrence of tubular aggregates. These two factors are not sufficient, however, since hormonal alteration by male castration nearly completely prevents the expression of tubular aggregates. Parallels are drawn with how expression of tubular aggregates is limited largely to males in diverse acquired and hereditary human neuromuscular disorders.
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Supported in part by NIH grants 5-K07-NS-00734 and 1-R01-NS23719, and by the Jay Slotkin Fund for Neuromuscular Research. Dr. Kuncl is the recipient of a Teacher Investigator Development Award from the NINCDS.
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Kuncl, R.W., Pestronk, A., Lane, J. et al. The MRL +/+ mouse: a new model of tubular aggregates which are gender- and age-related. Acta Neuropathol 78, 615–620 (1989). https://doi.org/10.1007/BF00691288
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DOI: https://doi.org/10.1007/BF00691288