Nematode homologue of PQBP1, a mental retardation causative gene, is involved in lipid metabolism.
basic_science · Level V
Where this comes from
- Record sourced from PubMed, PMID 19119319.
- Also identified by DOI 10.1371/journal.pone.0004104 and PMC identifier 2606030.
- Licence recorded as CC BY.
- The licence permits redistribution, so the abstract is shown in full and the full text is available from the publisher.
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.
Medical subject headings
- Caenorhabditis elegans
- Caenorhabditis elegans Proteins
- Carrier Proteins
- Lipid Metabolism
- X-Linked Intellectual Disability
- Nuclear Proteins