The X-chromosome-linked intellectual disability protein PQBP1 is a component of neuronal RNA granules and regulates the appearance of stress granules

• DOI: 10.1093/hmg/ddr430
• Publication Date: 2011-09-21T01:39:38Z
• Authors: S. A. Kunde; L. Musante; A. Grimme; U. Fischer; E. Muller; E. E. Wanker; V. M. Kalscheuer
• Keywords: Stress granule

The polyglutamine-binding protein 1 (PQBP1) has been linked to several X-linked intellectual disability disorders and progressive neurodegenerative diseases. While it is currently known that PQBP1 localizes in nuclear speckles engaged transcription splicing, we have now identified a cytoplasmic pool of PQBP1. Analysis complexes revealed six novel interacting proteins, namely the RNA-binding proteins KSRP, SFPQ/PSF, DDX1 Caprin-1, two subunits intracellular transport-related dynactin complex, p150Glued p27. complex formation dependent on presence RNA. Immunofluorescence studies primary neurons, co-localizes with its interaction partners specific granules, which stained positive for Our results suggest plays role mRNA metabolism. This further supported by partial co-localization fragile X mental retardation (FMRP), one best-studied found RNA granules. In studies, show arsenite-induced oxidative stress caused relocalization granules (SGs), where new binding as well FMRP. Additional indicated cellular distribution SG assembly. Together these data demonstrate modulation SGs involvement transport neuronal are critical importance development maintenance networks, thus illuminating route aberrations might influence cognitive function.