Details
- Autor(en) / Beteiligte
- Titel
- Nematode homologue of PQBP1, a mental retardation causative gene, is involved in lipid metabolism
- Ist Teil von
- PloS one, 2009, Vol.4 (1), p.e4104-e4104
- Ort / Verlag
- United States: Public Library of Science
- Erscheinungsjahr
- 2009
- Link zum Volltext
- Quelle
- EZB Electronic Journals Library
- Beschreibungen/Notizen
- 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. 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.
- Sprache
- Englisch
- Identifikatoren
- ISSN: 1932-6203eISSN: 1932-6203DOI: 10.1371/journal.pone.0004104Titel-ID: cdi_plos_journals_1289629023
- Format
- –
- Schlagworte
- Adipocytes, Adipocytes - cytology, Adipocytes - metabolism, Adipose Tissue, White - cytology, Analysis, Animals, Animals, Genetically Modified, Caenorhabditis elegans, Caenorhabditis elegans - anatomy & histology, Caenorhabditis elegans - physiology, Caenorhabditis elegans Proteins - genetics, Caenorhabditis elegans Proteins - metabolism, Carrier Proteins - genetics, Carrier Proteins - metabolism, Cell proliferation, Cells, Cultured, Developmental stages, Drosophila, Fatty acids, Genetic aspects, Genetic engineering, Genetics and Genomics/Disease Models, Genomes, Homology, Humans, Huntingtons disease, Insects, Intellectual disabilities, Intestine, Intestines - cytology, Larvae, Lipid metabolism, Lipid Metabolism - genetics, Lipids, Medical research, Mental illness, Mental retardation, Mental Retardation, X-Linked - genetics, Metabolism, Mutants, Nematoda, Nematodes, Neurodegeneration, Neurological Disorders/Developmental and Pediatric Neurology, Neuropathology, Nuclear Proteins - genetics, Nuclear Proteins - metabolism, Nutrition/Obesity, Patients, Pharmaceutical sciences, Physiological aspects, Physiology, Protein expression, Proteins, Recombinant Fusion Proteins - genetics, Recombinant Fusion Proteins - metabolism, RNA polymerase, Rodents, Syndrome, Transgenic animals, Triglycerides, Venus, Womens health