The Anti-PQBP1 Antibody, Rabbit Polyclonal from MyBioSource.com is a Rabbit Polyclonal antibody to PQBP1. This antibody recognizes Human antigen. The Anti-PQBP1 Antibody, Rabbit Polyclonal has been shown to work in the following applications: Immunocytochemistry, Immunofluorescence, and Western Blot.
Description
Polyglutamine tract-binding protein 1 (PQBP1) is an intrinsically disordered protein composed of a small folded WW domain and a long disordered region. PQBP1 binds to spliceosomal proteins WBP11 and U5-15kD through its N-terminal WW domain and C-terminal region, respectively. PQBP1 is an RNA-splicing factor that, when mutated, in humans causes Renpenning syndrome, an X-linked intellectual disability disease characterized by severe cognitive impairment, but also by physical defects that suggest PQBP1 has broader functions in embryonic development. PQBP1 interacts with splicing-related factors via the disordered/denatured domain and is involved in transcriptional and post-transcriptional regulation of gene expression in animals, and possibly also in plants. PQBP1 in bone marrow mesenchymal stem cells may play a crucial role in bone formation and cartilage development