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RENT1 / UPF1

UPF1 regulator of nonsense transcripts homolog (yeast)

RNA-dependent helicase and ATPase required for nonsense-mediated decay (NMD) of mRNAs containing premature stop codons. Is recruited to mRNAs upon translation termination and undergoes a cycle of phosphorylation and dephosphorylation; its phosphorylation appears to be a key step in NMD. Recruited by release factors to stalled ribosomes together with the SMG1C protein kinase complex to form the transient SURF (SMG1-UPF1-eRF1-eRF3) complex. In EJC-dependent NMD, the SURF complex associates with the exon junction complex (EJC) (located 50-55 or more nucleotides downstream from the termination codon) through UPF2 and allows the formation of an UPF1-UPF2-UPF3 surveillance complex which is believed to activate NMD. Phosphorylated UPF1 is recognized by EST1B/SMG5, SMG6 and SMG7 which are thought to provide a link to the mRNA degradation machinery involving exonucleolytic and endonucleolytic pathways, and to serve as adapters to protein phosphatase 2A (PP2A), thereby triggering UPF1 dephosphorylation and allowing the recycling of NMD factors. UPF1 can also activate NMD without UPF2 or UPF3, and in the absence of the NMD-enhancing downstream EJC indicative for alternative NMD pathways. Plays a role in replication-dependent histone mRNA degradation at the end of phase S; the function is independent of UPF2. For the recognition of premature termination codons (PTC) and initiation of NMD a competitive interaction between UPF1 and PABPC1 with the ribosome-bound release factors is proposed. The ATPase activity of UPF1 is required for disassembly of mRNPs undergoing NMD. Essential for embryonic viability.

Gene Name: UPF1 regulator of nonsense transcripts homolog (yeast)
Synonyms: UPF1, Delta helicase, HUPF1, PNORF1, KIAA0221, UP Frameshift 1, Yeast Upf1p homolog, ATP-dependent helicase RENT1, NORF1, RENT1, Smg-2
Target Sequences: NM_002911 NP_002902.2 Q92900

Publications (2)

1
Two molecular pathways (NMD and ERAD) contribute to a genetic epilepsy associated with the GABA(A) receptor GABRA1 PTC mutation, 975delC, S326fs328X. Kang JQ, Shen W, Macdonald RL. The Journal of neuroscience : the official journal of the Society for Neuroscience. 2009 29:2833-44. [PubMed:19261879] [PMC:PMC2687144]
2
The intronic GABRG2 mutation, IVS6+2T->G, associated with childhood absence epilepsy altered subunit mRNA intron splicing, activated nonsense-mediated decay, and produced a stable truncated 2 subunit. Tian M, Macdonald RL. The Journal of neuroscience : the official journal of the Society for Neuroscience. 2012 32:5937-52. [PubMed:22539854] [PMC:PMC3357398]

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RENT1 / UPF1 (4)
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GST, N-terminus (1)
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RENT1 / UPF1 Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
122.9 kDa
100 µg/$494
RENT1 / UPF1 Protein - 12.5% SDS-PAGE Stained with Coomassie Blue.
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Wheat Germ Extract
GST, N-terminus
10 µg/$479; 25 µg/$670
RENT1 / UPF1 Protein - Purified recombinant protein UPF1 was analyzed by SDS-PAGE gel and Coomassie Blue Staining
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HEK 293 Cells
Myc-DDK (Flag)
122.9 kDa
20 µg/$1,107
RENT1 / UPF1 Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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293T Cells
Myc-DDK (Flag)
122.9 kDa
20 µg/$150
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The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).