Reference - Detail
| RRC ID | 6456 |
|---|---|
| Author | Takahashi K, Yoshina S, Masashi M, Ito W, Inoue T, Shiwaku H, Arai H, Mitani S, Okazawa H. |
| Title | Nematode homologue of PQBP1, a mental retardation causative gene, is involved in lipid metabolism. |
| Journal | PLoS One |
| 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 the most frequent symptoms associating with PQBP1-linked MR patients. |
| Volume | 4(1) |
| Pages | e4104 |
| Published | 2009-1-1 |
| DOI | 10.1371/journal.pone.0004104 |
| PMID | 19119319 |
| PMC | PMC2606030 |
| MeSH | Adipocytes / cytology Adipocytes / metabolism Adipose Tissue, White / cytology Animals Animals, Genetically Modified Caenorhabditis elegans / anatomy & histology Caenorhabditis elegans / physiology* Caenorhabditis elegans Proteins / genetics Caenorhabditis elegans Proteins / metabolism* Carrier Proteins / genetics Carrier Proteins / metabolism* Cells, Cultured DNA-Binding Proteins Humans Intestines / cytology Lipid Metabolism / genetics* Mental Retardation, X-Linked / genetics* Nuclear Proteins / genetics Nuclear Proteins / metabolism* Recombinant Fusion Proteins / genetics Recombinant Fusion Proteins / metabolism Syndrome |
| IF | 2.74 |
| Times Cited | 16 |
| WOS Category | BIOCHEMISTRY & MOLECULAR BIOLOGY |
| Resource | |
| C.elegans | tm3004 tm3045 |