Adenosine deaminase (ADA)

Modality

Adenosine Deaminase (ADA)

Therapeutic Use of Adenosine Deaminase (ADA)

ADA-SCID is a severe combined immunodeficiency (SCID) subtype caused by inherited deficiency of adenosine deaminase (ADA). ADA deficiency is primarily linked to abnormalities in the development and function of lymphoids. Enzyme replacement therapy (ERT) has been approved for the treatment of ADA-SCID.

A bovine intestinal-derived ADA enzyme (Pegademase, Adagen) was introduced in 2007 with limitations in safety and available quantity. In 2018, Elapegademase-lvlr (Revcovi), a recombinant adenosine deaminase (rADA), was developed by Leadiant Biosciences and authorized by the FDA to treat patients with ADA-SCID.

The recombinant ADA (rADA) enzyme in Elapegademase-lvlr (Revcovi) is based on the bovine amino acid sequence and recombinantly expressed in Escherichia coli (E. coli). To prolong its half-life in vivo, rADA is covalently conjugated to mPEG with a succinimidyl carbamate linker and forms the active substance of Revcovi, SC-PEG rADA.

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Adenosine deaminase (ADA) Pipeline

Generic Name	Brand Name/ Alternative Name	Expression System	Indications	Manufacturer	R&D Stage
Elapegademase	Revcovi, レブコビ, PEG-rADA	E. coli	ADA- SCID: Adenosine deaminase deficiency (ADA) associated severe combined immunodeficiency disease (SCID)	Leadiant Biosciences, Teijin Pharma	Approval
Pegademase bovine	Adagen, PEG-ADA	Not applicable (derived from bovine intestine)	ADA- SCID	Chiesi Farmaceutici	Approval

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Adenosine deaminase (ADA)

Modality