4.4 Article

Disturbed apolipoprotein A-I-containing lipoproteins in fish-eye disease are improved by the lecithin:cholesterol acyltransferase produced by gene-transduced adipocytes in vitro

期刊

MOLECULAR GENETICS AND METABOLISM
卷 102, 期 2, 页码 229-231

出版社

ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.ymgme.2010.10.009

关键词

Lecithin:cholesterol acyltransferase; Fish-eye disease; Familial LCAT deficiency; Apolipoprotein A-I; Protein replacement therapy

资金

  1. Health and Labour Sciences Research Grants for Translational Research, Japan

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We report the in vitro efficacy of recombinant LCAT produced by lcat gene-transduced proliferative adipocytes (ccdPA/lcat), which has been developed for enzyme replacement therapy. ApoA-I-specific immunodetection in combination with 1D and 2D gel electrophoreses showed that the disturbed high-density lipoprotein subpopulation profile was clearly ameliorated by the in vitro incubation with ccdPA/lcat-derived recombinant LCAT. Thus, these results using ccdPA/lcat strongly suggest the cell implantation could contribute the enzyme replacement for the patients with LCAT deficiency. (C) 2010 Elsevier Inc. All rights reserved.

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