Nematode Homologue of PQBP1, a Mental Retardation Causative Gene,Is Involved in Lipid Metabolism |
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| Authors: | Keiko Takahashi Sawako Yoshina Maekawa Masashi Wakana Ito Takao Inoue Hiroki Shiwaku Hiroyuki Arai Shohei Mitani Hitoshi Okazawa |
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| Affiliation: | 1. Department of Neuropathology, Medical Research Institute, Tokyo Medical and Dental University, Tokyo, Japan.; 2. Department of Physiology, Tokyo Women''s Medical University School of Medicine, Tokyo, Japan.; 3. Department of Health Chemistry, Graduate School of Pharmaceutical Sciences, The University of Tokyo, Tokyo, Japan.;Massachusetts Institute of Technology, United States of America |
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| Abstract: | BackgroundPQBP1 is a causative gene for X-linked mental retardation (MR) whose patients frequently show lean body. C. elegans has a strictly conserved homologue gene of PQBP1, T21D12.3.Methodology and Principal FindingsWe generated Venus-transgenic and T21D12.3-mutant nematodes to analyze developmental expression patterns and in vivo functions of the nematode PQBP1 homologue protein (pqbp-1.1). During development, pqbp-1.1 is expressed from cell proliferation stage to larva stage. In larva, intestinal cells show the highest expression of pqbp-1.1, while it decreases in adult worms. The mutants of pqbp-1.1 show a decrease of the lipid content in intestinal cells. Especially, incorporation of fatty acid into triglyceride is impaired. ShRNA-mediated repression of PQBP1 also leads to reduction of lipid content in mammalian primary white adipocytes.Conclusion/ SignificanceThese results suggest that pqbp-1.1 is involved in lipid metabolism of intestinal cells. Dysfunction of lipid metabolism might underlie lean body, one of the most frequent symptoms associating with PQBP1-linked MR patients. |
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