Pegademase bovine Drugbank ID DB 00061 Protein chemical

Pegademase bovine Drugbank ID : DB 00061 Protein chemical formula : C 1821 H 2834 N 484 O 552 S 14 Protein average weight : 40788. 2000

Description: Bovine adenosine deaminase derived from bovine intestine that has been extensively pegylated for extended serum half life. Indication: For treatment of adenosine deaminase deficiency. Pharmacodynamics: Used to replace deficient or inactive adenosine deaminase which leads to severe combined immunodeficiency disease (SCID). The enzyme is responsible for converting adenosine to inosine. In the absence of adenosine deaminase, the purine substrates adenosine, 2' deoxyadenosine and their metabolites are actually toxic to lymphocytes thereby leading to diminished immune function. Mechanism of action: Pegademase converts adenosine (toxic) to inosine (less toxic) by deamination. It also converts 2' deoxyadenosine to 2' deoxyinosine via deamination. Categories: Enzyme Replacement Agents. Affected oragnism: Humans and other mammals

Sequence: MAQTPAFNKPKVELHVHLDGAIKPETILYYGRKRGIALPADTPEELQNIIGMDKPLSLPEFLA KFDYYMPAIAGCREAVKRIAYEFVEMKAKDGVVYVEVRYSPHLLANSKVEPIPWNQAEGD LTPDEVVSLVNQGLQEGERDFGVKVRSILCCMRHQPSWSSEVVELCKKYREQTVVAIDLAG DETIEGSSLFPGHVKAYAEAVKSGVHRTVHAGEVGSANVVKEAVDTLKTERLGHGYHTLED ATLYNRLRQENMHFEVCPWSSYLTGAWKPDTEHPVVRFKNDQVNYSLNTDDPLIFKSTLD TDYQMTKNEMGFTEEEFKRLNINAAKSSFLPEDEKKELLDLLYKAYGMPSPASAEQCL Targets : Adenosine, Growth factor receptor bound protein 2

BRANDS : Adagen Company : Enzon Inc. Chemical name : (monomethoxypolyethylene glycol succinimidyl) 11 17 adenosine deaminase [CH 3 (OCH 2)x O (C=O) CH 2 (C=O) NH 2]y adenosine deamninase, where x = 114 oxyetylene groups per PEG strand y= 11 17 primary amino groups of lysine onto which succinyl PEG is attached. Description: Adagen is a modified enzyme. It is a conjugate of numerous strands of monomethoxypolyethylene glycol (PEG), molecular weight 5, 000, covalently attached to the enzyme adenosine deaminase (ADA). ADA (adenosine deaminase EC 3. 5. 4. 4) used in the manufacture of ADAGEN®(pegademase bovine) Injection is derived from bovine intestine. It works by correcting the levels of ADA in the body, which improves immune system function. Used For/Prescribed for : It is used for Treating severe combined immunodeficiency disease (SCID) in certain patients with adenosine deaminase (ADA) deficiency.

Formulation : Each ml of ADAGEN injection contains, 250 units of Pegademase bovine, !. 20 mg of Monobasic sodium phoshphate, USP, 5. 58 mg of Dibasic sodium phoshphate, USP, 8. 50 mg of Sodiium chloride, USP and q. s. to !. 0 ml of water for injection. Form : isotonic, pyrogen free, sterile solution, p. H 7. 2 7. 4 Route of administration : intramusular injection Dosage : The dosage of ADAGEN® (pegademase bovine) Injection should be individualized. The recommended dosing schedule is 10 U/kg for the first dose, 15 U/kg for the second dose, and 20 U/kg for the third dose. The usual maintenance dose is 20 U/kg per week. Further increases of 5 U/kg/week may be necessary, but a maximum single dose of 30 U/kg should not be exceeded. Contraindication : allergic

Side effects : SEVERE side effects occur: Severe allergic reactions (rash; hives; itching; difficulty breathing; tightness in the chest; swelling of the mouth, face, lips, or tongue); dark urine; severe or persistent tiredness or weakness; unusual bruising or bleeding. • Most COMMON side effects: Headache; pain or redness at the injection site. Drug Interaction : A total of 1 drugs (2 brand generic names) are known to interact mopderately with Adagen (pegademase bovine) which are Krystexxa (pegloticase) and pegloticase

Refrence : http: //www. accessdata. fda. gov/drugsatfda_docs/label/2008/01981 8 s 042 lbl. pdf http: //www. adagen. com/ http: //www. drugs. com/dosage/adagen. html