Immunocytochemical localization of a full-length myotonin protein kinase in rat L6 myoblasts.
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.
Saitoh
N
N
Institute of Molecular and Cellular Biosciences, University of Tokyo, Japan.
Sasagawa
N
N
Koike
H
H
Shimokawa
M
M
Sorimachi
H
H
Ishiura
S
S
Suzuki
K
K
D000818:Animals; D002460:Cell Line / Q000737:chemistry / Q000201:enzymology; D018076:DNA, Complementary; D005455:Fluorescent Antibody Technique; D006801:Humans; D018482:Muscle, Skeletal / Q000166:cytology* / Q000201:enzymology*; D016296:Mutagenesis / Q000502:physiology; D065636:Myotonin-Protein Kinase; D011494:Protein Kinases / Q000032:analysis* / Q000235:genetics; D017346:Protein Serine-Threonine Kinases; D051381:Rats; D014162:Transfection
C585517:DMPK protein, human; D018076:DNA, Complementary; D011494:Protein Kinases; D065636:Myotonin-Protein Kinase; D017346:Protein Serine-Threonine Kinases