title: Strain- and age-dependent loss of sarcoglycan complex in cardiomyopathic hamster hearts and its re-expression by delta-sarcoglycan gene transfer in vivo.
abstract: The delta-sarcoglycan (SG) gene is deleted in hamsters with hereditary cardiomyopathies. Immunological analyses of heart before, but not after, the progression of cardiomyopathy (CM) revealed that the BIO 14.6 strain, a model of hypertrophic CM, heterogeneously preserved alpha- and gamma-SG with loss of beta- and delta-SG. In contrast, the TO-2 strain, a model of dilated CM, did not show either SG. Furthermore, in vivo transfer of the full length delta-SG gene to TO-2 hearts expressed all four SGs. Thus, this age- and strain-dependent features suggest a more feasible setting for TO-2 than BIO 14.6 to verify both CM progression and the efficacy of gene therapy.
authors.affiliation: Division of Pharmacy, Niigata University Medical Hospital, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Kawada
authors.forename: Y
authors.initials: Y
authors.lastname: Nakatsuru
authors.forename: A
authors.initials: A
authors.lastname: Sakamoto
authors.forename: T
authors.initials: T
authors.lastname: Koizumi
authors.forename: W S
authors.initials: WS
authors.lastname: Shin
authors.forename: Y
authors.initials: Y
authors.lastname: Okai-Matsuo
authors.forename: J
authors.initials: J
authors.lastname: Suzuki
authors.forename: Y
authors.initials: Y
authors.lastname: Uehara
authors.forename: M
authors.initials: M
authors.lastname: Nakazawa
authors.forename: H
authors.initials: H
authors.lastname: Sato
authors.forename: T
authors.initials: T
authors.lastname: Ishikawa
authors.forename: T
authors.initials: T
authors.lastname: Toyo-oka
mesh_terms: D000367:Age Factors; D000818:Animals; D009202:Cardiomyopathies / Q000235:genetics* / Q000628:therapy; D006224:Cricetinae; D003598:Cytoskeletal Proteins / Q000235:genetics* / Q000378:metabolism; D004195:Disease Models, Animal; D049030:Dystroglycans; D016189:Dystrophin / Q000032:analysis; D015870:Gene Expression; D018014:Gene Transfer Techniques; D015316:Genetic Therapy; D015151:Immunoblotting; D007150:Immunohistochemistry; D008297:Male; D008562:Membrane Glycoproteins / Q000235:genetics* / Q000378:metabolism; D008647:Mesocricetus; D009206:Myocardium / Q000166:cytology / Q000378:metabolism; D049031:Sarcoglycans; D014162:Transfection
chemical_list: D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008562:Membrane Glycoproteins; D049031:Sarcoglycans; D049030:Dystroglycans