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 humoraland cellular immune responses due to the lack of adenosine deaminase (ADA) enzyme. PEG-ADA therapy tries tocounteract ADA deficiency by conjugates conformed of numerous mono-methoxy polyethyleneglycol chains linkednon-covalently, and ADA enzymes, which are bound by lysine residues. PEG-ADA protects from any proteolyticattack, and presentation of antigens, increasing their lifespan within the organism. Enzyme replacement therapy withPEGylated ADA provides metabolic correction and improvement in immune function and clinical parameters. Itseffectiveness is confirmed by the increase of B and T lymphocytes in questionable time ranges.

Año de publicación:

2019

Keywords:

• Severe combined immune deficiency (SCID)
• Adenosine deaminase deficiency (ADA)
• Pegademasebovine (PEG-ADA)
• vasculitis

Fuente:

scopus