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Identification
- Summary
Pegademase is a modified form of bovine adenosine deaminase used to treat adenosine deaminase deficiency, a condition which leads to the formation of adenosine metabolites which are cytotoxic to lymphocytes.
- Generic Name
- Pegademase
- DrugBank Accession Number
- DB00061
- Background
Bovine adenosine deaminase derived from bovine intestine that has been extensively pegylated for extended serum half life.
- Type
- Biotech
- Groups
- Approved
- Biologic Classification
- Protein Based Therapies
Other protein based therapies - Protein Structure
- Protein Chemical Formula
- C1821H2834N484O552S14
- Protein Average Weight
- 40788.2 Da
- Sequences
>DB00061 sequence MAQTPAFNKPKVELHVHLDGAIKPETILYYGRKRGIALPADTPEELQNIIGMDKPLSLPE FLAKFDYYMPAIAGCREAVKRIAYEFVEMKAKDGVVYVEVRYSPHLLANSKVEPIPWNQA EGDLTPDEVVSLVNQGLQEGERDFGVKVRSILCCMRHQPSWSSEVVELCKKYREQTVVAI DLAGDETIEGSSLFPGHVKAYAEAVKSGVHRTVHAGEVGSANVVKEAVDTLKTERLGHGY HTLEDATLYNRLRQENMHFEVCPWSSYLTGAWKPDTEHPVVRFKNDQVNYSLNTDDPLIF KSTLDTDYQMTKNEMGFTEEEFKRLNINAAKSSFLPEDEKKELLDLLYKAYGMPSPASAE QCL
Download FASTA Format- Synonyms
- (monomethoxypolyethylene glycol succinimidyl) 11-17-adenosine deaminase
- PEG-adenosine deaminase
- pegADA
- Pegademase
- Pegademase bovine
Pharmacology
- Indication
For treatment of adenosine deaminase deficiency
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Indication Type Indication Combined Product Details Approval Level Age Group Patient Characteristics Dose Form Management of Adenosine deaminase deficiency •••••••••••• •••• •••••• ••••••••••••••• ••••••••••• •••••• •••••••• •••••••••••••••• •••••••• ••••••••• - Contraindications & Blackbox Warnings
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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.
Target Actions Organism AAdenosine metabolizerHumans UGrowth factor receptor-bound protein 2 binderHumans - Absorption
Time to peak for plasma adenosine deaminase is 2 to 3 days
- Volume of distribution
Not Available
- Protein binding
Not Available
- Metabolism
- Not Available
- Route of elimination
Not Available
- Half-life
plasma adenosine deaminase elimination half-life is 3 to >6 days
- Clearance
Not Available
- Adverse Effects
Improve decision support & research outcomesWith structured adverse effects data, including: blackbox warnings, adverse reactions, warning & precautions, & incidence rates. View sample adverse effects data in our new Data Library!Improve decision support & research outcomes with our structured adverse effects data.- Toxicity
Not Available
- Pathways
- Not Available
- Pharmacogenomic Effects/ADRs
- Not Available
Interactions
- Drug Interactions
- This information should not be interpreted without the help of a healthcare provider. If you believe you are experiencing an interaction, contact a healthcare provider immediately. The absence of an interaction does not necessarily mean no interactions exist.
Drug Interaction Integrate drug-drug
interactions in your softwareAntihemophilic factor (recombinant), PEGylated The therapeutic efficacy of Antihemophilic factor (recombinant), PEGylated can be decreased when used in combination with Pegademase. Certolizumab pegol The therapeutic efficacy of Certolizumab pegol can be decreased when used in combination with Pegademase. Damoctocog alfa pegol The therapeutic efficacy of Damoctocog alfa pegol can be decreased when used in combination with Pegademase. Elapegademase The therapeutic efficacy of Elapegademase can be decreased when used in combination with Pegademase. Lipegfilgrastim The therapeutic efficacy of Lipegfilgrastim can be decreased when used in combination with Pegademase. - Food Interactions
- Not Available
Products
Drug product information from 10+ global regionsOur datasets provide approved product information including:dosage, form, labeller, route of administration, and marketing period.Access drug product information from over 10 global regions.- Brand Name Prescription Products
Name Dosage Strength Route Labeller Marketing Start Marketing End Region Image Adagen Injection, solution 250 [iU]/1mL Intramuscular Leadiant Biosciences, Inc. 2010-10-18 2019-06-30 US 
Categories
- ATC Codes
- L03AX04 — Pegademase
- Drug Categories
- Chemical TaxonomyProvided by Classyfire
- Description
- Not Available
- Kingdom
- Organic Compounds
- Super Class
- Organic Acids
- Class
- Carboxylic Acids and Derivatives
- Sub Class
- Amino Acids, Peptides, and Analogues
- Direct Parent
- Peptides
- Alternative Parents
- Not Available
- Substituents
- Not Available
- Molecular Framework
- Not Available
- External Descriptors
- Not Available
- Affected organisms
- Humans and other mammals
Chemical Identifiers
- UNII
- HW3H7D91F6
- CAS number
- 130167-68-9
References
- Synthesis Reference
Paul Skonezny, "Process for preparing dideoxyinosine using adenosine deaminase enzyme." U.S. Patent US20040175804, issued September 09, 2004.
US20040175804- General References
- Not Available
- External Links
- UniProt
- P56658
- Genbank
- AF280603
- PubChem Substance
- 46508490
- 59768
- ChEMBL
- CHEMBL1201514
- PharmGKB
- PA450831
- RxList
- RxList Drug Page
- Drugs.com
- Drugs.com Drug Page
- Wikipedia
- Adenosine_deaminase
Clinical Trials
- Clinical Trials
Clinical Trial & Rare Diseases Add-on Data Package
Explore 4,000+ rare diseases, orphan drugs & condition pairs, clinical trial why stopped data, & more. Preview package Phase Status Purpose Conditions Count Start Date Why Stopped 100+ additional columns Unlock 175K+ rows when you subscribe.View sample data3 Completed Treatment ADA-SCID / Adenosine Deaminase Deficiency / Severe Combined Immunodeficiency Syndrome 1 somestatus stop reason just information to hide
Pharmacoeconomics
- Manufacturers
- Sigma tau pharmaceuticals inc
- Packagers
- Ben Venue Laboratories Inc.
- Enzon Inc.
- Dosage Forms
Form Route Strength Injection, solution Intramuscular 250 [iU]/1mL - Prices
Unit description Cost Unit Adagen 250 unit/ml vial 4375.0USD ml DrugBank does not sell nor buy drugs. Pricing information is supplied for informational purposes only.- Patents
- Not Available
Properties
- State
- Liquid
- Experimental Properties
Property Value Source hydrophobicity -0.428 Not Available isoelectric point 5.33 Not Available
Targets
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Yes
Metabolizer
References
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [Article]
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [Article]
- Van Linden A, Eltzschig HK: Role of pulmonary adenosine during hypoxia: extracellular generation, signaling and metabolism by surface adenosine deaminase/CD26. Expert Opin Biol Ther. 2007 Sep;7(9):1437-47. [Article]
- Kemeny-Beke A, Jakab A, Zsuga J, Vecsernyes M, Karsai D, Pasztor F, Grenczer M, Szentmiklosi AJ, Berta A, Gesztelyi R: Adenosine deaminase inhibition enhances the inotropic response mediated by A1 adenosine receptor in hyperthyroid guinea pig atrium. Pharmacol Res. 2007 Aug;56(2):124-31. Epub 2007 May 10. [Article]
- Mohsenin A, Mi T, Xia Y, Kellems RE, Chen JF, Blackburn MR: Genetic removal of the A2A adenosine receptor enhances pulmonary inflammation, mucin production, and angiogenesis in adenosine deaminase-deficient mice. Am J Physiol Lung Cell Mol Physiol. 2007 Sep;293(3):L753-61. Epub 2007 Jun 29. [Article]
- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Unknown
- Actions
- Binder
- General Function
- Non-enzymatic adapter protein that plays a pivotal role in precisely regulated signaling cascades from cell surface receptors to cellular responses, including signaling transduction and gene expression (PubMed:11726515, PubMed:37626338). Thus, participates in many biological processes including regulation of innate and adaptive immunity, autophagy, DNA repair or necroptosis (PubMed:35831301, PubMed:37626338, PubMed:38182563). Controls signaling complexes at the T-cell antigen receptor to facilitate the activation, differentiation, and function of T-cells (PubMed:9489702, PubMed:36864087). Mechanistically, engagement of the TCR leads to phosphorylation of the adapter protein LAT, which serves as docking site for GRB2 (PubMed:9489702). In turn, GRB2 establishes a a connection with SOS1 that acts as a guanine nucleotide exchange factor and serves as a critical regulator of KRAS/RAF1 leading to MAPKs translocation to the nucleus and activation (PubMed:12171928, PubMed:25870599). Functions also a role in B-cell activation by amplifying Ca(2+) mobilization and activation of the ERK MAP kinase pathway upon recruitment to the phosphorylated B-cell antigen receptor (BCR) (PubMed:25413232, PubMed:29523808). Plays a role in switching between autophagy and programmed necrosis upstream of EGFR by interacting with components of necrosomes including RIPK1 and with autophagy regulators SQSTM1 and BECN1 (PubMed:35831301, PubMed:38182563). Regulates miRNA biogenesis by forming a functional ternary complex with AGO2 and DICER1 (PubMed:37328606). Functions in the replication stress response by protecting DNA at stalled replication forks from MRE11-mediated degradation. Mechanistically, inhibits RAD51 ATPase activity to stabilize RAD51 on stalled replication forks (PubMed:38459011). Additionally, directly recruits and later releases MRE11 at DNA damage sites during the homology-directed repair (HDR) process (PubMed:34348893)
- Specific Function
- ephrin receptor binding
- Gene Name
- GRB2
- Uniprot ID
- P62993
- Uniprot Name
- Growth factor receptor-bound protein 2
- Molecular Weight
- 25206.21 Da
References
- Ramos-Morales F, Dominguez A, Rios RM, Barroso SI, Infante C, Schweighoffer F, Tocque B, Pintor-Toro JA, Tortolero M: Adenosine deaminase is a specific partner for the Grb2 isoform Grb3-3. Biochem Biophys Res Commun. 1997 Aug 28;237(3):735-40. [Article]
Drug created at June 13, 2005 13:24 / Updated at August 02, 2024 07:21