Anti-UPF3B from rabbit

Regulator of nonsense transcripts 3B (UniProt: Q9BZI7; also known as Nonsense mRNA reducing factor 3B, Up-frameshift suppressor 3 homolog B, UPF3B, hUpf3B, Up-frameshift suppressor 3 homolog on chromosome X, hUpf3p-X) is encoded by the UPF3B (also known as RENT3B, UPF3X) gene (Gene ID: 65109) in human. UPF3B is widely expressed, including in testis, uterus, heart, muscle, brain, spinal cord, and placenta. It shuttles between the nucleus and the cytoplasm. It is involved in nonsense-mediated decay (NMD) of mRNAs containing premature stop codons by associating with the nuclear exon junction complex (EJC) and serves as a link between the EJC core and NMD machinery. UPF3B recruits UPF2 at cytoplasmic side of the nuclear envelope and the subsequent formation of an UPF1-UPF2-UPF3 surveillance complex is believed to activate NMD. In cooperation with UPF2 it stimulates both ATPase and RNA helicase activities of UPF1. Mutations in UPF3B gene have been linked to X-linked mental retardation that is characterized by significantly low general intellectual functioning associated with impairments in adaptive behavior, autistic features, poor musculature and other abnormalities. Two isoforms of UPF3B have been described that are produced by alternative splicing.