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Human acid maltase‐deficient myogenic cell transformation with origin‐defective SV40: Characterization of a cloned line
Author(s) -
Usuki Fusako,
Higuchi Itsuro,
Soejima Yasuko,
Hattori Masakazu,
Maruyama Ikuro,
Osame Mitsuhiro
Publication year - 1991
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880140308
Subject(s) - myogenesis , clone (java method) , multinucleate , biology , transfection , cell culture , maltase , myocyte , microbiology and biotechnology , cell growth , cellular differentiation , biochemistry , enzyme , gene , genetics
Abstract A clonal human skeletal muscle cell line showing acid maltase deficiency (AMD) was established through the transfection of origin‐defective SV40 DNA. The low acid α‐glucosidase activity and glycogenosomes in this clone corresponded to AMD. This clone, in spite of loading glycogenosomes, was competent not only as to proliferation without contact inhibition but also as to myogenic differentiation to some extent. Dexamethasone promoted the formation by the transformant of multinucleated myotubes, which expressed acetylcholine receptors. The existence of glycogenosomes did not seem to affect the proliferation or differentiation of myoblasts. The aberrant acid α‐glucosidase expressed in the transformed myogenic clone was shown to be biochemically identical to that in AMD fibroblasts. This transformant should be of great value for investigating the pathogenesis of AMD because of the possibility of supplying semi‐permanently a uniform myogenic cell line expressing AMD.