|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
While paroxysmal nocturnal hemoglobinuria (PNH) results from the combined deficiency of the regulatory complement proteins CD55 and CD59, which is caused by somatic mutation of a common membrane anchor, isolated CD55 or CD59 deficiency is associated with the CHAPLE syndrome and polyneuropathy, respectively.
|
31421540 |
2019 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
Flow cytometry revealed deficiency of the glycophosphatidylinositol (GPI)-anchored complement regulatory proteins, CD59 and CD55, and he was diagnosed with PNH.
|
30262533 |
2018 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
PNH's etiopathogenesis is based on acquired mutations that lead to the reduction or absence of CD55 and CD59 complement regulators, which are responsible for some of the disease's major clinical features, like intravascular hemolysis, cytopenias and thrombosis.
|
29486674 |
2018 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal hematopoietic disease caused by expansion of a stem cell that harbors a somatic mutation in <i>PIGA</i> PIGA mutant blood cells are deficient in the complement regulator proteins CD55 and CD59, making them susceptible to intravascular hemolysis due to a failure to regulate the APC on erythrocytes.
|
30504334 |
2018 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
CD16/CD66b detected 16 (25.8%) additional patients over CD55/CD59 (P<0.05) and was more sensitive in detecting the PNH clone with higher negative predictive value.
|
29355143 |
2017 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
Despite the uniform deficiency of CD55 and of CD59, there are always two distinct populations of PNH RBCs, with (C3+) and without (C3-) C3 binding.
|
28629435 |
2017 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal nonneoplastic hematopoietic stem cell disease characterized by an acquired mutation of the PIG-A gene with reduction or absence of CD55 and CD59.
|
25688459 |
2015 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
Patients were screened for paroxysmal nocturnal hemoglobinuria by flow cytometry using anti-CD55 and anti-CD59 antibodies.
|
24969051 |
2014 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
PNH is due to a somatic, acquired mutation in the X-linked phosphatidylinositol glycan class A (PIG-A) gene, which impairs the membrane expression on affected blood cells of a number of proteins, including the complement regulators CD55 and CD59.
|
23402025 |
2013 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
Loss of CD55 and CD59 on erythrocytes causes complement-mediated lysis in paroxysmal nocturnal hemoglobinuria (PNH), a disease that manifests after clonal expansion of hematopoietic cells with somatic PIGA mutations.
|
22305531 |
2012 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
Peripheral blood from 489 recently diagnosed patients with aplastic anaemia (AA) and 316 with refractory anaemia (RA) of myelodysplastic syndrome was evaluated to characterize CD55(-)CD59(-) [paroxysmal nocturnal haemoglobinuria (PNH)]-type blood cells associated with bone marrow (BM) failure.
|
19656154 |
2009 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by absence of CD55 and CD59 from the surface of affected cells.
|
18158579 |
2008 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Paroxysmal nocturnal hemoglobinuria (PNH) is a hematopoietic disorder caused by PIGA mutations that lead to a loss of all glycosylphospatidylinositol (GPI)-anchored proteins including, CD55 and CD59.
|
15160958 |
2004 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
Complement-mediated hemolysis in PNH is explained by the deficiency of glycosylphosphatidylinositol (GPI)-anchored proteins, CD55 and CD59 on erythrocyte surfaces.
|
14972783 |
2002 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
Although many of the clinical manifestations (e.g., hemolytic anemia) of the disease can be explained by a deficiency of GPI-anchored complement regulatory proteins such as CD59 and CD55, it is unclear why the PNH clone dominates hematopoiesis and why it is prone to evolve into acute leukemia.
|
9238050 |
1997 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
In this study we measured two glycosylphosphatidylinositol (GPI)-linked molecules on platelets (CD55 and CD59) and two GPI-linked proteins on neutrophils (CD14 and CD16), comparing their expression on normal and PNH patients.
|
8885138 |
1996 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
Flow cytometry of erythrocytes using anti-CD59 or of granulocytes using either anti-CD55 or anti-CD59 provides the most accurate technique for the diagnosis of paroxysmal nocturnal hemoglobinuria; it is clearly more specific, more quantitative, and more sensitive than the tests for PNH that depend upon hemolysis by complement (the acidified serum lysis [Ham] test, the sucrose lysis test, and the complement lysis sensitivity [CLS] test).
|
8652849 |
1996 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
It is the deficiency of erythrocyte CD55 and CD59 that accounts for the intravascular hemolysis and hemoglobinuria that are the clinical hallmarks of PNH.
|
8843541 |
1996 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
Moreover, they lack surface expression of complement regulatory proteins such as DAF (CD55) and CD59, that are the most important glycosylphosphatidylinositol (GPI)-anchored membrane proteins defective in haemopoietic cells of patients with PNH.
|
7524616 |
1994 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
Analyses of greater than 98% surface DAF-negative PMN and MNC from a patient with PNH III erythrocytes showed precursor DAF protein approximately 3 kD smaller in each cell type than in normal cells.
|
1688570 |
1990 |
|
Paroxysmal nocturnal hemoglobinuria
|
0.100 |
Biomarker
|
disease |
BEFREE |
The results suggest that the primary molecular defect underlying the clinical manifestations of PNH may be the lack of the membrane-associated DAF protein and that the abnormal cells may also exhibit impaired CR1 function.
|
6225118 |
1983 |