regulator of nonsense transcripts 3B (nonsense mRNA reducing factor 3B, up-frameshift suppressor homolog 3B, hUpf3B, hUpf3p-X, UPF3B, RENT3B, UPF3X)

Function: 1) part of a post-splicing multiprotein complex with NXF1, RBM8A, RENT1, RENT2, RENT3A, RENT3B & RNPS 2) found in a complex with SMG1, RENT1, RENT2 & RENT3B 3) found in a mRNP complex with RENT3A, RENT3B, RBM8A, THOC4 & NXF1 4) found in a mRNA decay complex with EXOSC2, EXOSC4, EXOSC10, PARN, XRN1, DCP2, RENT1, RENT2 & RENT3B 2) involved in nonsense-mediated decay (NMD) of mRNAs containing premature stop codons 3) binds spliced mRNA upstream of exon-exon junctions 4) interacts with EST1A, RENT2 & RBM8A Compartment: - nucleus, cytoplasm - shuttles between the nucleus & cytoplasm Alternative splicing: named isoforms=2 Expression: - expressed in testis, uterus, prostate, heart, muscle, brain, spinal cord & placenta Pathology: - defects in UPF3B are the cause of X-linked syndromic mental retardation type 14 (MRXS14)

Properties

SIZE: MW = 58 kD entity length = 483 aa COMPARTMENT: cytoplasm cell nucleus MOTIF: binding site SITE: 30-255 FOR-BINDING-OF: regulator of nonsense transcripts 2 MOTIF: binding site SITE: 52-57 FOR-BINDING-OF: regulator of nonsense transcripts 2 binding site SITE: 430-447 FOR-BINDING-OF: RNA-binding protein 8A NMD

Database Correlations

OMIM 300298 UniProt Q9BZI7 Pfam PF03467

References

UniProt :accession Q9BZI7