nematode homologue of pqbp1, a mental retardation causative gene, is involved in lipid metabolismpqbp1线虫同系物,精神发育迟滞诱发基因,参与脂类代谢.pdfVIP

nematode homologue of pqbp1, a mental retardation causative gene, is involved in lipid metabolismpqbp1线虫同系物,精神发育迟滞诱发基因,参与脂类代谢.pdf

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nematode homologue of pqbp1, a mental retardation causative gene, is involved in lipid metabolismpqbp1线虫同系物,精神发育迟滞诱发基因,参与脂类代谢

Nematode Homologue of PQBP1, a Mental Retardation Causative Gene, Is Involved in Lipid Metabolism 1,2 2 3 1 3 1 Keiko Takahashi , Sawako Yoshina , Maekawa Masashi , Wakana Ito , Takao Inoue , Hiroki Shiwaku , 3 2 1 Hiroyuki Arai , Shohei Mitani , Hitoshi Okazawa * 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 Abstract Background: PQBP1 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 Findings: We 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/ Significance: These results suggest that pqbp-1.1 is involved in lipid metabolism of intestinal cells. Dysfunction of lipid metabolism might underlie lean body, one of

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