UPF3B Antibody, Biotin conjugated

Code CSB-PA883646LD01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) UPF3B Polyclonal antibody
Uniprot No.
Target Names
UPF3B
Alternative Names
UPF3B antibody; RENT3B antibody; UPF3X antibody; Regulator of nonsense transcripts 3B antibody; Nonsense mRNA reducing factor 3B antibody; Up-frameshift suppressor 3 homolog B antibody; hUpf3B antibody; Up-frameshift suppressor 3 homolog on chromosome X antibody; hUpf3p-X antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Regulator of nonsense transcripts 3B protein (319-423AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Biotin
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA
Protocols
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Involved in nonsense-mediated decay (NMD) of mRNAs containing premature stop codons by associating with the nuclear exon junction complex (EJC) and serving as link between the EJC core and NMD machinery. Recruits UPF2 at the cytoplasmic side of the nuclear envelope and the subsequent formation of an UPF1-UPF2-UPF3 surveillance complex (including UPF1 bound to release factors at the stalled ribosome) is believed to activate NMD. In cooperation with UPF2 stimulates both ATPase and RNA helicase activities of UPF1. Binds spliced mRNA upstream of exon-exon junctions. In vitro, stimulates translation; the function is independent of association with UPF2 and components of the EJC core.
Gene References into Functions
  1. The authors discovered that UPF3B (i) interacts with the release factors, (ii) delays translation termination and (iii) dissociates post-termination ribosomal complexes that are devoid of the nascent peptide. PMID: 28899899
  2. UPF3B gene mutation is associated with Lujan-Fryns syndrome. PMID: 26358559
  3. the neurodevelopmental phenotype of UPF3B missense mutation is caused by impairment of nonsense-mediated mRNA decay pathway function altering neuronal differentiation. PMID: 26012578
  4. These findings indicate that SATB2 activates UPF3B expression through binding to its promoter. PMID: 23925499
  5. Data indicate the mutation p.R430X of UPF3B gene as the genetic etiology in the mental retardation pedigree. PMID: 22957832
  6. results demonstrate that the UPF3B-dependent NMD pathway is a major regulator of the transcriptome and that its targets have important roles in neuronal cells. PMID: 22182939
  7. The two cases with renal dysplasia and developmental delay showed remarkable clinical variability despite having the same mutation in UPF3B. PMID: 22609145
  8. Our results demonstrate that in addition to Lujan-Fryns and FG syndromes, UPF3B protein truncation mutations can cause also nonspecific XLMR. PMID: 19238151
  9. 3.4 A resolution crystal structure of a minimal UPF3b-EJC assembly, consisting of the interacting domains of five proteins (UPF3b, MAGO, Y14, eIF4AIII, and Barentsz) together with RNA and adenylyl-imidodiphosphate PMID: 20479275
  10. A conserved domain of hUpf3b mediates an interaction with the EJC protein Y14. Y14 is required for nonsense-mediated decay induced by tethered hUpf3b. PMID: 12718880
  11. The protein region that mediates this interaction and discriminates between hUpf3a and hUpf3b in NMD function is located in the C-terminal domain and fully contained within a small sequence that is highly conserved in Upf3b but not Upf3a proteins PMID: 16601204
  12. UPF3B induces nonsense mediated decay in the cytoplasm PMID: 17194930
  13. Three mutations lead to the introduction of a premature termination codon and subsequent nonsense-mediated mRNA decay of mutant UPF3B mRNA. PMID: 17704778
  14. UPF2 and UPF3b cooperatively stimulate both ATPase and RNA helicase activities of UPF1. PMID: 18066079
  15. Results suggest 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
  16. binds to spliced mRNAs upstream of exon-exon junctions; is part of mRNP complexes that are ready for nuclear export and that participate in nonsense-mediated mRNA decay PMID: 11546873
  17. binds RNPS1 protein, part of the postsplicing complex deposited 5' to exon-exon junctions PMID: 11546874

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Involvement in disease
Mental retardation, X-linked, syndromic, 14 (MRXS14)
Subcellular Location
Nucleus. Cytoplasm. Note=Shuttling between the nucleus and the cytoplasm.
Protein Families
RENT3 family
Tissue Specificity
Expressed in testis, uterus, prostate, heart, muscle, brain, spinal cord and placenta.
Database Links

HGNC: 20439

OMIM: 300298

KEGG: hsa:65109

STRING: 9606.ENSP00000276201

UniGene: Hs.103832

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