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Poly-U binding splicing factor 60KDa

Fir, FBP-interacting repressor, RoBPI, PUF60, FUSE-binding protein-interacting repressor, FARP2
The protein encoded by this gene is a Ro RNP-binding protein. It interacts with Ro RNPs and their interaction is thought to represent a gain of function for Ro RNPs. This protein also forms a ternary complex with far upstream element (FUSE) and FUSE-binding protein. It can repress a c-myc reporter via the FUSE. It is also known to target transcription factor IIH and inhibit activated transcription. This gene is implicated in the xeroderma pigmentosum disorder. There are two alternatively spliced transcript variants of this gene encoding different isoforms. There seems to be evidence of multiple polyadenylation sites for this gene. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: c-Myc, ACID, V1a, POLYMERASE, CAN
Papers on Fir
First fetal case of the 8q24.3 contiguous genes syndrome.
New
Romana et al., Paris, France. In Am J Med Genet A, Jan 2016
This rare condition has to be considered as a contiguous genes syndrome because its phenotype is generated by the SCRIB and PUF60 adjacent gene endophenotypes.
Whole-exome sequencing and genome-wide methylation analyses identify novel disease associated mutations and methylation patterns in idiopathic hypereosinophilic syndrome.
New
Grønbæk et al., Copenhagen, Denmark. In Oncotarget, Jan 2016
These included the spliceosome gene PUF60 and the cadherin gene CDH17.
Exon-centric regulation of ATM expression is population-dependent and amenable to antisense modification by pseudoexon targeting.
New
Vorechovsky et al., Southampton, United Kingdom. In Sci Rep, Dec 2015
We show that a key nonsense-mediated RNA decay switch exon (NSE) in ATM is repressed by U2AF, PUF60 and hnRNPA1.
Endothelial RhoGEFs: A systematic analysis of their expression profiles in VEGF-stimulated and tumor endothelial cells.
New
Vázquez-Prado et al., Mexico. In Vascul Pharmacol, Nov 2015
Among them, we found that the most abundant endothelial RhoGEFs were: Tuba, FGD5, Farp1, ARHGEF17, TRIO, P-Rex1, ARHGEF15, ARHGEF11, ABR, Farp2, ARHGEF40, ALS, DOCK1, DOCK7 and DOCK6.
Concomitant expression of far upstream element (FUSE) binding protein (FBP) interacting repressor (FIR) and its splice variants induce migration and invasion of non-small cell lung cancer (NSCLC) cells.
New
Breuhahn et al., Heidelberg, Germany. In J Pathol, Nov 2015
Because over-expression of DNA-interacting far upstream element binding protein (FBP) supports non-small cell lung cancer (NSCLC) migration, we asked whether its repressor, FBP-interacting repressor (FIR) is functionally inactivated and how FIR might affect NSCLC cell biology.
The first echinoderm poly-U-binding factor 60 kDa (PUF60) from sea cucumber (Stichopus monotuberculatus): Molecular characterization, inducible expression and involvement of apoptosis.
New
Wang et al., Guangzhou, China. In Fish Shellfish Immunol, Nov 2015
Poly-U-binding factor 60 kDa (PUF60), also known as Ro RNA binding protein (RoBPI) and FBP interacting repressor (FIR), is a multifunctional protein that is involved in a variety of nuclear processes including pre-mRNA splicing, apoptosis and transcription regulation.
PUF60: a prominent new target of the autoimmune response in dermatomyositis and Sjögren's syndrome.
New
Casciola-Rosen et al., Redwood City, United States. In Ann Rheum Dis, Sep 2015
The targeted autoantigen was identified as poly(U)-binding-splicing factor 60 kDa (PUF60) using (i) a human protein array and (ii) two-dimensional gel electrophoresis and liquid chromatography tandem mass spectrometry peptide sequencing.
Identification of U2AF(35)-dependent exons by RNA-Seq reveals a link between 3' splice-site organization and activity of U2AF-related proteins.
New
Vorechovsky et al., Southampton, United Kingdom. In Nucleic Acids Res, May 2015
Exons upregulated in depleted cells were preceded by longer AG exclusion zones and PPTs than downregulated or control exons and were largely activated by PUF60 and repressed by CAPERα.
Gene therapy of c-myc suppressor FUSE-binding protein-interacting repressor by Sendai virus delivery prevents tracheal stenosis.
Shiotani et al., Tokorozawa, Japan. In Plos One, 2014
The objective of this study was to determine whether the Sendai virus (SeV)-mediated c-myc suppressor, a far upstream element (FUSE)-binding protein (FBP)-interacting repressor (FIR), modulates wound healing of the airway mucosa, and whether it prevents tracheal stenosis in an animal model of induced mucosal injury.
ClRTL1 Encodes a Chinese Fir RNase III-Like Protein Involved in Regulating Shoot Branching.
Shi et al., Nanjing, China. In Int J Mol Sci, 2014
A branching mutant of Chinese fir named "Dugansha" (Cunninghamia lanceolata var.
Global Reprogramming of Transcription in Chinese Fir (Cunninghamia lanceolata) during Progressive Drought Stress and after Rewatering.
Li et al., Beijing, China. In Int J Mol Sci, 2014
Chinese fir (Cunninghamia lanceolata), an evergreen conifer, is the most commonly grown afforestation species in southeast China due to its rapid growth and good wood qualities.
Identification of genes important for cutaneous function revealed by a large scale reverse genetic screen in the mouse.
Smyth et al., Melbourne, Australia. In Plos Genet, 2014
Many of these were not previously associated with skin disease in the organ (Mysm1, Vangl1, Trpc4ap, Nom1, Sparc, Farp2, and Prkab1), while others were ascribed new cutaneous functions on the basis of the screening approach (Krt76, Lrig1, Myo5a, Nsun2, and Nf1).
SAP155-mediated splicing of FUSE-binding protein-interacting repressor serves as a molecular switch for c-myc gene expression.
GeneRIF
Nomura et al., Chiba, Japan. In Mol Cancer Res, 2012
Data indicate that altered FIR and c-myc pre-mRNA splicing, in addition to c-Myc expression by augmented FIR/FIRDeltaexon2-SAP155 complex, potentially contribute to colorectal cancer development.
Integral roles of a guanine nucleotide exchange factor, FARP2, in osteoclast podosome rearrangements.
GeneRIF
Kumanogoh et al., Suita, Japan. In Faseb J, 2010
Findings reveal an integral role of FARP2 for regulation of Rac1 and integrin beta3 throughout podosome rearrangement in osteoclastogenesis.
Quantitative characterization of the interactions among c-myc transcriptional regulators FUSE, FBP, and FIR.
GeneRIF
Braddock et al., New Haven, United States. In Biochemistry, 2010
FIR is monomeric in solution but dimerizes upon DNA binding; DNA-induced dimerization is mediated by FIR's RNA recognition motif.
Common variants at 2q37.3, 8q24.21, 15q21.3 and 16q24.1 influence chronic lymphocytic leukemia risk.
Impact
Houlston et al., United Kingdom. In Nat Genet, 2010
We identified four new risk loci for CLL at 2q37.3 (rs757978, FARP2; odds ratio (OR) = 1.39;
Dimerization and protein binding specificity of the U2AF homology motif of the splicing factor Puf60.
GeneRIF
Sattler et al., Heidelberg, Germany. In J Biol Chem, 2009
Puf60-UHM binds to ULM sequences in the splicing factors SF1, U2AF65, and SF3b155.
Farp2 and Stk25 are candidate genes for the HDL cholesterol locus on mouse chromosome 1.
GeneRIF
Paigen et al., Bar Harbor, United States. In Arterioscler Thromb Vasc Biol, 2009
Confirmed Hdlq14 as a separate independent quantitative trait locus for HDL and narrowed the region to 2 genes, Farp2 and Stk25, with considerable evidence for both.
c-myc expression: keep the noise down!
Review
Levens et al., Bethesda, United States. In Mol Cells, 2005
Those features include the use of an expanded proximal promoter, the averaging of input from dozens of transcription factors, and real-time feedback using the supercoil-deformable Far UpStream Element (FUSE) as physical sensor of ongoing transcriptional activity, and the FUSE binding protein (FBP) as well as the FBP interacting repressor (FIR) as effectors to enforce normal transcription from the c-myc promoter.
Defective interplay of activators and repressors with TFIH in xeroderma pigmentosum.
Impact
Levens et al., Bethesda, United States. In Cell, 2001
Here, XPB and XPD mutations are shown to block transcription activation by the FUSE Binding Protein (FBP), a regulator of c-myc expression, and repression by the FBP Interacting Repressor (FIR).
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