What is CD55 and CD59?

Abstract. The regulatory proteins CD55 and CD59 are glycolsylphosphatidylinositol-anchored, type I cell surface proteins, which inhibit formation of the C3 convertases and prevent the terminal polymerization of the membrane attack complexes, respectively.

What is PNH flow cytometry?

Flow cytometry is the gold standard in diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) by detecting the absence of glycol-phosphatidyl inositol (GPI)-linked protein expression on granulocyte and monocyte surfaces.

What is the confirmatory test for PNH?

The main diagnostic test for individuals with suspected PNH is flow cytometry, a blood test that can identify PNH cells (blood cells that are missing GPI-anchored proteins).

What is the function of CD59?

Function. CD59 inhibits the formation of MAC pores in the membranes of expressing cells: it is a ‘suicide inhibitor’, locking onto C8 in the forming MAC to block the recruitment of C9 into the complex. In common with other GPI-linked proteins, cross-linking of CD59 can cause cell activation.

Where is CD59 located?

CD59 glycoprotein, also known as MAC-inhibitory protein (MAC-IP), membrane inhibitor of reactive lysis (MIRL), or protectin, is a protein that in humans is encoded by the CD59 gene….

CD59
Location (UCSC) Chr 11: 33.7 – 33.74 Mb Chr 2: 103.9 – 103.92 Mb
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What is flow cytometry technique?

Flow Cytometry is a technique used to detect and measure physical and chemical characteristics of a population of cells or particles. In this process, a sample containing cells or particles is suspended in a fluid and injected into the flow cytometer instrument.

What is CD59 deficiency?

CD59 deficiency is a common finding in RBCs and WBCs in patients with chronic hemolysis suffering from paroxysmal nocturnal hemoglobinuria in which the acquired mutation in the PIGA gene leads to membrane loss of glycosylphosphatidylinositol-anchored membrane proteins, including CD59.

What is MDS and PNH?

Acquired aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH) are pathogenically related nonmalignant bone marrow failure disorders linked to T-cell-mediated autoimmunity; they are associated with an increased risk of secondary myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML).

What is the importance of CD59?

CD59 is responsible for binding complement components C8 and C9 and preventing formation of membrane attack complex to protect cells from complement damage. Individuals who lack CD59 are typically ill with neuropathy, strokes, and episodes of Coombs-negative hemolysis.