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 what is the exact problem in PNH?  



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Author4 Posts
  #1

What is the exact problem in PNH, defect in CD59, or PIG-A (Phosphatidyl inositol glycan A), or decay accelerating factor (DAF)?

Confused! Please clearify! :shock:




  #2

The exact defect in Paroxysmal Nocturnal Hemoglobinuria (PNA) is a non-inherited (aquired) mutation on the gene responsiblle for the synhtesis of the enzyme PIG-A (phosphatidylinositol glycan class A) in the X chromosome of some stem cells.

This PIG-A is involved in the synthesis of the glycosyl-phosphatidylinositol (GPI) anchor which binds some complement-regulating surface proteins on all hematopoietic cell membranes (like DAF and CD59) and protect the cells from complement atack.

no enzyme > no anchor > no protection > hemolysis


  #3

Thank you very much! smiling face


  #4

"prep4usmle" wrote:
The exact defect in Paroxysmal Nocturnal Hemoglobinuria (PNA) is a non-inherited (aquired) mutation on the gene responsiblle for the synhtesis of the enzyme PIG-A (phosphatidylinositol glycan class A) in the X chromosome of some stem cells.

This PIG-A is involved in the synthesis of the glycosyl-phosphatidylinositol (GPI) anchor which binds some complement-regulating surface proteins on all hematopoietic cell membranes (like DAF and CD59) and protect the cells from complement atack.

no enzyme > no anchor > no protection > hemolysis


I understood maybe 50% of those words........





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