UPF3B Antibody
Description
What is UPF3B Antibody?
UPF3B antibodies are reagents designed to target the UPF3B protein, a component of the NMD machinery that degrades mRNAs containing premature termination codons (PTCs). These antibodies enable researchers to investigate UPF3B's expression, localization, and interactions in cellular contexts .
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Key Functions of UPF3B:
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Facilitates NMD by bridging exon junction complexes (EJCs) with downstream effectors like UPF2 and UPF1 .
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Regulates immune gene expression in olfactory sensory neurons (OSNs) and maintains olfactory receptor diversity .
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Modulates endoplasmic reticulum (ER) stress by interacting with IRE1α, impacting ER-associated degradation .
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Role of UPF3B in NMD and Disease
UPF3B is essential for RNA surveillance, with mutations linked to X-linked intellectual disability (XLID) and neurodevelopmental disorders .
Table 1: UPF3B Antibody Applications
UPF3B in Olfactory and Immune Regulation
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Olfactory Sensory Neurons (OSNs):
UPF3B loss disrupts OSN subsets and upregulates antimicrobial genes, suggesting a role in innate immunity . -
ER Stress Modulation:
UPF3B inhibits IRE1α kinase activity, linking NMD to ER stress adaptation .
Functional Redundancy with UPF3A
UPF3A compensates for UPF3B loss in NMD, maintaining mRNA surveillance in UPF3B-deficient cells .
Validation and Technical Considerations
Product Specs
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Target Background
- Research has demonstrated that UPF3B (i) interacts with release factors, (ii) delays translation termination, and (iii) disassembles post-termination ribosomal complexes lacking the nascent peptide. PMID: 28899899
- Mutations in the UPF3B gene have been linked to Lujan-Fryns syndrome. PMID: 26358559
- Studies suggest that the neurodevelopmental phenotype associated with UPF3B missense mutations arises from impaired nonsense-mediated mRNA decay pathway function, leading to altered neuronal differentiation. PMID: 26012578
- Data indicate that SATB2 activates UPF3B expression by binding to its promoter. PMID: 23925499
- Evidence suggests that the p.R430X mutation in the UPF3B gene is the genetic basis for mental retardation in a specific pedigree. PMID: 22957832
- Research findings indicate that the UPF3B-dependent NMD pathway plays a major role in regulating the transcriptome, and its targets have significant functions in neuronal cells. PMID: 22182939
- Two cases with renal dysplasia and developmental delay exhibited notable clinical variability despite having the same mutation in UPF3B. PMID: 22609145
- Results demonstrate that, in addition to Lujan-Fryns and FG syndromes, UPF3B protein truncation mutations can also cause nonspecific XLMR. PMID: 19238151
- A 3.4 Angstrom resolution crystal structure of a minimal UPF3b-EJC assembly has been determined. This assembly comprises the interacting domains of five proteins (UPF3b, MAGO, Y14, eIF4AIII, and Barentsz) along with RNA and adenylyl-imidodiphosphate. PMID: 20479275
- A conserved domain within hUpf3b mediates an interaction with the EJC protein Y14. Y14 is essential for nonsense-mediated decay induced by tethered hUpf3b. PMID: 12718880
- The protein region that mediates this interaction and differentiates between hUpf3a and hUpf3b in NMD function is located in the C-terminal domain and is fully contained within a small sequence that is highly conserved in Upf3b but not Upf3a proteins. PMID: 16601204
- UPF3B induces nonsense-mediated decay in the cytoplasm. PMID: 17194930
- Three mutations result in the introduction of a premature termination codon, leading to subsequent nonsense-mediated mRNA decay of mutant UPF3B mRNA. PMID: 17704778
- UPF2 and UPF3b work cooperatively to stimulate both ATPase and RNA helicase activities of UPF1. PMID: 18066079
- Research suggests that UPF3A levels are tightly regulated by a post-transcriptional switch to maintain appropriate levels of NMD substrates in cells containing different levels of UPF3B. PMID: 19503078
- UPF3B binds to spliced mRNAs upstream of exon-exon junctions; it is part of mRNP complexes that are prepared for nuclear export and participate in nonsense-mediated mRNA decay. PMID: 11546873
- UPF3B binds to RNPS1 protein, a component of the postsplicing complex deposited 5' to exon-exon junctions. PMID: 11546874
Q&A
FAQs for UPF3B Antibody in Academic Research
Advanced Research Questions
How do UPF3B and UPF3A redundantly regulate NMD, and how can this be experimentally disentangled?
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Functional redundancy analysis:
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Perform co-depletion experiments (UPF3A + UPF3B siRNA/KO) to assess transcriptome-wide NMD substrate accumulation via RNA-Seq .
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Use UPF3B mutants lacking UPF2-binding domains (Δ2BD) to isolate UPF3A-specific contributions .
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Rescue assays: Transfect UPF3B/UPF3A domain-swap constructs into dKO cells to map functional regions .
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What are the implications of UPF3B-UPF1 interaction dynamics in disease models?
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Experimental framework:
How does UPF3B-CASC3 interaction influence EJC-dependent NMD?
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Mechanistic studies:
Data Tables
Table 1: Key UPF3B Antibody Validation Approaches
Table 2: UPF3B vs. UPF3A Functional Redundancy Evidence
Methodological Recommendations
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For co-IP studies, include RNase treatment controls to distinguish RNA-dependent/independent interactions .
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In murine models, combine antibody validation with transcriptome-wide analysis (e.g., RNA-Seq in Upf3a KO mESCs) .
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For clinical correlations, use patient-derived cells with UPF3B mutations to assess antibody utility in diagnostic workflows .
