Enzyme Replacement therapy with Pegademase Bovine for Human Adenosine Deaminase Primary Immunodeficiency

Abstract

Adenosine deaminase deficiency (ADA) represents an immune system disorder producing abnormalities in humoral and cellular immune responses due to the lack of adenosine deaminase (ADA) enzyme. PEG-ADA therapy tries to counteract ADA deficiency by conjugates conformed of numerous mono-methoxy polyethyleneglycol chains linked non-covalently, and ADA enzymes, which are bound by lysine residues. PEG-ADA protects from any proteolytic attack, and presentation of antigens, increasing their lifespan within the organism. Enzyme replacement therapy with PEGylated ADA provides metabolic correction and improvement in immune function and clinical parameters. Its effectiveness is confirmed by the increase of B and T lymphocytes in questionable time ranges.