Skip to Content
MilliporeSigma
  • Dystrophin conferral using human endothelium expressing HLA-E in the non-immunosuppressive murine model of Duchenne muscular dystrophy.

Dystrophin conferral using human endothelium expressing HLA-E in the non-immunosuppressive murine model of Duchenne muscular dystrophy.

Human molecular genetics (2010-10-16)
Chang-Hao Cui, Shunichiro Miyoshi, Hiroko Tsuji, Hatsune Makino, Seiichi Kanzaki, Daisuke Kami, Masanori Terai, Harumi Suzuki, Akihiro Umezawa
ABSTRACT

Human leukocyte antigen (HLA)-E is a non-classical major histocompatibility complex class I (Ib) molecule, which plays an important role in immunosuppression. In this study, we investigated the immunomodulating effect of HLA-E in a xenogeneic system, using human placental artery-derived endothelial (hPAE) cells expressing HLA-E in a mouse model. In vitro cell lysis analysis by primed lymphocytes in combination with siRNA transfection showed that HLA-E is necessary for inhibition of the immune response. Similarly, in vivo cell implantation analysis with siRNA-mediated down-regulation of HLA-E demonstrates that HLA-E is involved in immunosuppression. As hPAE cells efficiently transdifferentiate into myoblasts/myocytes in vitro, we transplanted the cells into mdx mice, a model of Duchenne muscular dystrophy. hPAE cells conferred dystrophin to myocytes of the 'immunocompetent' mdx mice with extremely high efficiency. These findings suggest that HLA-E-expressing cells with a myogenic potential represent a promising source for cell-based therapy of patients with muscular dystrophy.

MATERIALS
Product Number
Brand
Product Description

Sigma-Aldrich
Endothelial Cell Characterization Kit, Endothelial cell characterization kits enable researchers to characterize cultured endothelial cells in both a constitutive & activated state using basic ICC techniques.