|
Hemophagocytic Lymphohistiocytosis, Familial, 1
|
0.300 |
Biomarker
|
disease |
GENOMICS_ENGLAND |
|
|
|
|
Lyme Disease
|
0.070 |
Biomarker
|
disease |
BEFREE |
Although several PFam54 members play an important role in the pathogenesis of Lyme disease, the exact function has been determined only for CspA, which binds complement regulator factor H (CFH) and factor H-like protein 1 (FHL-1); thus, CspA is essential to resist the vertebrate host's immune response.
|
31204045 |
2019 |
|
Lyme Disease
|
0.070 |
Biomarker
|
disease |
BEFREE |
Identification and characterization of the factor H and FHL-1 binding complement regulator-acquiring surface protein 1 of the Lyme disease spirochete Borrelia spielmanii sp. nov.
|
18706858 |
2009 |
|
Lyme Disease
|
0.070 |
Biomarker
|
disease |
BEFREE |
BBA68 (BbCRASP-1) of the Lyme disease spirochetes binds human factor H (FH) and FH-like protein 1 (FHL-1).
|
16622245 |
2006 |
|
Lyme Disease
|
0.070 |
Biomarker
|
disease |
BEFREE |
Functional characterization of BbCRASP-2, a distinct outer membrane protein of Borrelia burgdorferi that binds host complement regulators factor H and FHL-1.
|
16925556 |
2006 |
|
Lyme Disease
|
0.070 |
Biomarker
|
disease |
BEFREE |
It has been demonstrated that Borrelia hermsii, a causative agent of relapsing fever, produces a factor H (FH) and FH-like protein 1 (FHL-1) binding protein.
|
16861638 |
2006 |
|
Lyme Disease
|
0.070 |
Biomarker
|
disease |
BEFREE |
In the present study we have elucidated the putative potential of other members of the gbb54 paralogous family, including orthologs ZSA66, ZSA69, ZSA70, ZSA71, ZSA72 and ZSA73 of the European B. burgdorferi s.s. strain ZS7, to bind human FHL-1 and factor H. In spite of their overall similarity in protein sequence, between 47% and 67%, and the fact that the C-terminal region of ZSA69 shows 70% similarity with BbCRASP-1, none of the orthologous proteins was able to bind human FHL-1 and/or factor H. BbCRASP-1 is the only member of the paralogous gene family gbb54 that binds to human complement regulators, supporting the notion that BbCRASP-1 plays a critical role in evasion of complement by B. burgdorferi s.s. and thus may be helpful in the development of novel therapeutic strategies against Lyme borreliosis.
|
17160605 |
2006 |
|
Lyme Disease
|
0.070 |
AlteredExpression
|
disease |
BEFREE |
Complement resistance of Borrelia burgdorferi correlates with the expression of BbCRASP-1, a novel linear plasmid-encoded surface protein that interacts with human factor H and FHL-1 and is unrelated to Erp proteins.
|
14607842 |
2004 |
|
Age related macular degeneration
|
0.060 |
GeneticVariation
|
disease |
BEFREE |
A sensitivity analysis of genetic variants known to be associated with late stage AMD showed that rs1061170 (p.Y402H) in the complement factor H (CFH) gene influences the association of Cer d18:1/16:0 with GA. To understand the possible influence of this genetic variant on ceramide levels, we established a cell-based assay to test the modulation of genes in the ceramide metabolism by factor H-like protein 1 (FHL-1), an alternative splicing variant of CFH that also harbors the 402 residue.
|
30071029 |
2018 |
|
Age related macular degeneration
|
0.060 |
GeneticVariation
|
disease |
BEFREE |
PTX3 was found to be present in the macula of donor eyes and the AMD-associated Y402H polymorphism altered the binding of FHL-1 to PTX3.
|
29374201 |
2018 |
|
Age related macular degeneration
|
0.060 |
Biomarker
|
disease |
BEFREE |
AMD-associated lipid deposition in BrM decreases FHL-1 diffusion.
|
29312308 |
2017 |
|
Age related macular degeneration
|
0.060 |
GeneticVariation
|
disease |
BEFREE |
FHL-1 is largely bound to Bruch's membrane through interactions with heparan sulfate, and we show that the common Y402H polymorphism in the CFH gene, associated with an increased risk of AMD, reduces the binding of FHL-1 to this heparan sulfate.
|
25305316 |
2014 |
|
Age related macular degeneration
|
0.060 |
GeneticVariation
|
disease |
BEFREE |
The FH Y402H polymorphism associated with AMD causes a reduction in binding of FH and FHL-1 to CRP and M protein.
|
18263814 |
2008 |
|
Age related macular degeneration
|
0.060 |
Biomarker
|
disease |
BEFREE |
In addition, we identify the Factor H-like protein 1 (FHL-1), an alternative splice product of the CFH gene as an additional protein that includes the risk residue 402, and thus confers risk for AMD.
|
17399790 |
2007 |
|
Rheumatoid Arthritis
|
0.020 |
Biomarker
|
disease |
BEFREE |
This study shows a novel protective and anti-inflammatory role of the two important complement regulators FHL-1 and factor H in rheumatoid arthritis and suggests a disease controlling role of the two proteins.
|
12780697 |
2003 |
|
Rheumatoid Arthritis
|
0.020 |
AlteredExpression
|
disease |
BEFREE |
Different regulation of factor H and FHL-1/reconectin by inflammatory mediators and expression of the two proteins in rheumatoid arthritis (RA).
|
10931160 |
2000 |
|
Glycogen storage disease type II
|
0.010 |
Biomarker
|
disease |
BEFREE |
AMD-associated lipid deposition in BrM decreases FHL-1 diffusion.
|
29312308 |
2017 |
|
Hemolytic-Uremic Syndrome
|
0.010 |
Biomarker
|
disease |
BEFREE |
In conclusion, multiple interactions of key complement inhibitors FH, FHR-1 and FHL-1 with Stx2 corroborate our hypothesis of a direct role of complement in EHEC-associated HUS.
|
24317278 |
2014 |
|
Actinic keratosis
|
0.010 |
AlteredExpression
|
disease |
BEFREE |
Analysis of CFH and FHL-1 expression in vivo in invasive cSCCs (n=65), in situ cSCCs (n=38), and premalignant lesions (actinic keratoses, n=37) by immunohistochemistry showed that they were specifically expressed by tumor cells in cSCCs and the staining intensity was stronger in cSCCs than in in situ cSCCs and actinic keratoses.
|
23938460 |
2014 |
|
Neoplasms
|
0.010 |
AlteredExpression
|
group |
BEFREE |
The expression of CFH and FHL-1 mRNAs was also significantly higher in cSCC tumors (n=6) than in normal skin (n=11).
|
23938460 |
2014 |
|
Squamous cell carcinoma of skin
|
0.010 |
Biomarker
|
disease |
BEFREE |
These results provide evidence for a role of CFH and FHL-1 in cSCC progression and identify them as progression markers and potential therapeutic targets in SCCs of skin.
|
23938460 |
2014 |
|
Drusen
|
0.010 |
Biomarker
|
disease |
BEFREE |
We also show that FHL-1 is retained in drusen whereas FH coats the periphery of the lesions, perhaps inhibiting their clearance.
|
25305316 |
2014 |
|
Glomerulonephritis
|
0.010 |
Biomarker
|
disease |
BEFREE |
We also showed that FHL-1 (factor H-like protein 1) was present in the patient with aHUS and absent in the patient with glomerulonephritis.
|
23870792 |
2013 |
|
Complement Factor H Deficiency
|
0.010 |
Biomarker
|
disease |
BEFREE |
This study emphasizes that modifiers such as CD46 and FHL-1 may determine the kidney phenotype of patients who present with homozygous CFH deficiency.
|
23870792 |
2013 |
|
Atypical Hemolytic Uremic Syndrome
|
0.010 |
Biomarker
|
disease |
BEFREE |
We also showed that FHL-1 (factor H-like protein 1) was present in the patient with aHUS and absent in the patient with glomerulonephritis.
|
23870792 |
2013 |