Immunocytochemical localization of myotonin protein kinase on muscle from patients with congenital myotonic dystrophy

Abstract

Using a polyclonal anti myotonin-protein kinase (M-PK) antibody against synthetic M-PK peptides corresponding to part of the amino acid sequence, and the immunohistochemical analysis of indirect immunoperoxidase, we have investigated localization of M-PK on muscle from patients with congenital myotonic dystrophy. In congenital myotonic dystrophy (MD) patients, one month and 3 months old, M-PK was weakly expressed at sarcolemma of muscle fibers. In congenital MD patients from 2 to 9 years of age, M-PK was clearly expressed at sarcolemma of muscle fibers. M-PK of immature muscle is weakly expressed at sarcolemma. With aging, M-PK is clearly expressed at sarcolemma of muscle from MD patient and normal control.