Differentiation potential of primary myogenic cells derived from skeletal muscle of dystonia musculorum mice.

The dystonia musculorum (dt) mouse has a mutation in the gene encoding the cytoskeletal crosslinker protein bullous pemphigoid antigen 1 (Bpag1). These mice have perturbations in the cytoarchitecture of skeletal muscle. Bpag1 has been hypothesized to be involved in the maintenance rather than the establishment of the muscle cell architecture given that cytoskeletal disruptions are observed in the muscle tissue of post-natal dt mice. Not known is whether Bpag1-deficiency affects the proliferative and differentiation potential of myogenic cells. In the present investigation, we show that the growth rate of cultured primary myogenic cells derived from dt mice, as assessed by BrdU incorporation, is similar to that of myogenic cells derived from wild-type littermates. The myogenic differentiation potential of dt versus wild-type cells was monitored by examining the expression of myosin heavy chain by immunofluorescence, and by analyzing the expression profiles of myogenic regulatory factors and myogenic differentiation markers by RT-PCR. In all instances, both dt and wild-type myogenic cells displayed a similar differentiation profile. Furthermore, the absence of any observable differences in the proliferation and differentiation rates of dt and wild-type cells was not due to an overexpression of plectin, another crosslinker protein, in dt cells. Together, these findings demonstrate that the early phases of myogenic differentiation occur independently of Bpag1.