Strain- and age-dependent loss of sarcoglycan complex in cardiomyopathic hamster hearts and its re-expression by delta-sarcoglycan gene transfer in vivo.
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.
Kawada
T
T
Division of Pharmacy, Niigata University Medical Hospital, Japan.
Nakatsuru
Y
Y
Sakamoto
A
A
Koizumi
T
T
Shin
W S
WS
Okai-Matsuo
Y
Y
Suzuki
J
J
Uehara
Y
Y
Nakazawa
M
M
Sato
H
H
Ishikawa
T
T
Toyo-oka
T
T
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
D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008562:Membrane Glycoproteins; D049031:Sarcoglycans; D049030:Dystroglycans