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Complement factor H-related 1

HFL-1, H36, CFHR1, FHR-1
This gene encodes a secreted protein belonging to the complement factor H protein family. It binds to Pseudomonas aeruginosa elongation factor Tuf together with plasminogen, which is proteolytically activated. It is proposed that Tuf acts as a virulence factor by acquiring host proteins to the pathogen surface, controlling complement, and facilitating tissue invasion. Mutations in this gene are associated with an increased risk of atypical hemolytic-uremic syndrome. [provided by RefSeq, Oct 2009] (from NCBI)
Top mentioned proteins: CAN, Complement Factor H, HAD, V1a, AGE
Papers on HFL-1
Factor H inhibits complement activation induced by liposomal and micellar drugs and the therapeutic antibody rituximab in vitro.
Józsi et al., Budapest, Hungary. In Nanomedicine, Jan 2016
The FH-related protein CFHR1 had no inhibitory effect.
Metformin attenuates gefitinib-induced exacerbation of pulmonary fibrosis by inhibition of TGF-β signaling pathway.
He et al., Chongqing, China. In Oncotarget, Jan 2016
We found that in lung HFL-1 fibroblast cells, TGF-β or conditioned medium from TKI-treated lung cancer PC-9 cells or conditioned medium from TKI-resistant PC-9GR cells, induced significant fibrosis, as shown by increased expression of Collegen1a1 and α-actin, while metformin inhibited expression of fibrosis markers.
Heterogeneity but individual constancy of epitopes, isotypes and avidity of factor H autoantibodies in atypical hemolytic uremic syndrome.
Józsi et al., Madrid, Spain. In Mol Immunol, Jan 2016
FH autoantibodies from FHR-1 deficient patients (n=13) mainly recognized FH, its SCR19-20 fragment and FHR-1, but autoantibody specificity in patients who are homo- or heterozygous for the CFHR1 gene (n=6) was heterogeneous.
Synthesis, Anticancer Activity, and Genome Profiling of Thiazolo Arene Ruthenium Complexes.
Therrien et al., Cluj-Napoca / Kolozsvár, Romania. In J Med Chem, Dec 2015
All complexes were tested in vitro for their antiproliferative activity on three tumor cell lines (HeLa, A2780, and A2780cisR) and on a noncancerous cell line (HFL-1).
Radiosensitization of non-small cell lung cancer by kaempferol.
Yao et al., Taipei, Taiwan. In Oncol Rep, Nov 2015
However, the same doses did not affect HFL1 normal lung cell growth.
Comparison of Mouse and Human Retinal Pigment Epithelium Gene Expression Profiles: Potential Implications for Age-Related Macular Degeneration.
Bergen et al., Amsterdam, Netherlands. In Plos One, 2014
For example, CFHR1, most likely the main complement regulator in AMD pathogenesis was highly expressed in human RPE, but almost absent in mouse RPE.
Optimization of in vitro measurement of proteasome activity in mammalian cells using fluorogenic substrates.
Gonos et al., Greece. In Free Radic Biol Med, 2014
Herein, we describe an optimization procedure for the measurement of CT-L, T-L and C-L activities in cell lysates of fibroblasts (HFL-1), melanocytes (B16F10) and peripheral blood mononuclear cells (PBMCs) using fluorogenic peptide substrates in a mid-throughput 96-well plate format.
Role of complement in enterohemorrhagic Escherichia coli-Induced hemolytic uremic syndrome.
Würzner et al., Innsbruck, Austria. In Semin Thromb Hemost, 2014
Both proteins also compete with FH for Stx binding, so that in the presence of FHR-1 less FH is bound to Stx and therefore more is available for endothelial cell protection.
Complement factor H related proteins (CFHRs).
Roumenina et al., Jena, Germany. In Mol Immunol, 2014
Factor H related proteins comprise a group of five plasma proteins: CFHR1, CFHR2, CFHR3, CFHR4 and CFHR5, and each member of this group binds to the central complement component C3b.
Atypical haemolytic uraemic syndrome with underlying glomerulopathies. A case series and a review of the literature.
Buzio et al., Parma, Italy. In Nephrol Dial Transplant, 2013
The following tests were performed in all six patients: serum C3 and C4 levels, ADAMTS13 activity, CFH levels and anti-CFH autoantibodies and genetic screening for CFH, MCP, CFI, C3 and CFHR1-3 mutations and risk haplotypes associated with aHUS.
Complement regulator-acquiring surface proteins of Borrelia burgdorferi: Structure, function and regulation of gene expression.
Stevenson et al., Frankfurt am Main, Germany. In Ticks Tick Borne Dis, 2013
One key borrelial immune escape mechanism involves the inactivation of host complement attack through acquisition of human immune regulators factor H (CFH), factor H-like protein 1 (FHL1), factor H-related protein 1 (CFHR1), CFHR2, and/or CFHR5.
Atypical hemolytic uremic syndrome-associated variants and autoantibodies impair binding of factor h and factor h-related protein 1 to pentraxin 3.
Józsi et al., Jena, Germany. In J Immunol, 2012
we show that native factor H, factor H-like protein 1, and factor H-related protein 1 (CFHR1) bind to PTX3
A hybrid CFHR3-1 gene causes familial C3 glomerulopathy.
Pickering et al., London, United Kingdom. In J Am Soc Nephrol, 2012
A hybrid CFHR3-1 gene causes familial C3 glomerulopathy.
Factor H and CFHR1 polymorphisms associated with atypical Haemolytic Uraemic Syndrome (aHUS) are differently expressed in Tunisian and in Caucasian populations.
Sánchez-Corral et al., Monastir, Tunisia. In Int J Immunogenet, 2012
Reduced expression of the CFHR1 allele has been associated with higher risk to atypical Haemolytic Uraemic Syndrome in Spanish patients.
Relevance of complement factor H-related 1 (CFHR1) genotypes in age-related macular degeneration.
Spanish Multicenter Group on AMD et al., Madrid, Spain. In Invest Ophthalmol Vis Sci, 2012
Analysis of the CFHR1 genotypes provide sufficient information to delineate the individual risk of developing age-related macular degeneration.
Comprehensive analysis of Copy Number Variation of genes at chromosome 1 and 10 loci associated with late age related macular degeneration.
Baird et al., Melbourne, Australia. In Plos One, 2011
A significant association with deletion of CFHR1-4 was identified in patients who presented with bilateral geographic atrophy.
Genetic mechanisms and age-related macular degeneration: common variants, rare variants, copy number variations, epigenetics, and mitochondrial genetics.
Tuo et al., Bethesda, United States. In Hum Genomics, 2011
Copy number variations in regulators of complement activation genes (CFHR1 and CFHR3) and glutathione S transferase genes (GSTM1 and GSTT1) have been associated with AMD, and several additional loci have been identified as regions of potential interest but require further evaluation.
Genome-wide association study identifies susceptibility loci for IgA nephropathy.
Lifton et al., New York City, United States. In Nat Genet, 2011
We identified three independent loci in the major histocompatibility complex, as well as a common deletion of CFHR1 and CFHR3 at chromosome 1q32 and a locus at chromosome 22q12 that each surpassed genome-wide significance (P values for association between 1.59 × 10⁻²⁶ and 4.84 × 10⁻⁹ and minor allele odds ratios of 0.63-0.80).
A common CFH haplotype, with deletion of CFHR1 and CFHR3, is associated with lower risk of age-related macular degeneration.
Chakravarthy et al., Belfast, United Kingdom. In Nat Genet, 2006
A common haplotype was associated with decreased risk of AMD. This haplotype carried a deletion of CFHR1 and CFHR3, and the proteins encoded by these genes were absent in serum of homozygotes.
Evidence for a common evolutionary origin of inverted repeat transposons in Drosophila and plants: hobo, Activator, and Tam3.
Gelbart et al., Cambridge, United States. In Cell, 1991
We have sequenced HFL1 from D. melanogaster, the only cloned hobo element shown to have transposase activity.
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