PMID: 8945766Nov 8, 1996Paper

Immunocytochemical localization of a full-length myotonin protein kinase in rat L6 myoblasts

Neuroscience Letters
N SaitohK Suzuki

Abstract

Expansion mutation of CTG-repeat motifs within myotonin protein kinase (MtPK) gene is responsible for pathological changes in myotonic dystrophy (DM). To explore its pathological role in skeletal muscle, a full-length human MtPK cDNA was transfected into rat L6 myogenic cell line. Recombinantly expressed human MtPK protein in L6 cell line has a predicted molecular mass of 70 kDa. We have raised a polyclonal antibody against a synthetic peptide in the deduced sequence of the C-terminal portion of MtPK. MtPK in L6 cell is localized to perinuclear region, that resembles with sarcoplasmic reticulum. The MtPK-transfected myoblast cells established in this study will allow us to elucidate the molecular pathomechanism of muscle manifestations in DM.

References

Jun 1, 1989·Brain : a Journal of Neurology·C J HöwelerA Staal
May 1, 1995·The Journal of Clinical Investigation·J P MounseyJ R Moorman
Jun 6, 1995·Proceedings of the National Academy of Sciences of the United States of America·L TimchenkoC T Caskey
Nov 1, 1993·Human Molecular Genetics·P F van der VenH F Epstein
Mar 1, 1993·Human Molecular Genetics·M S MahadevanJ Lamerdin

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