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    Elapegademase

    ACTIVE PHARMA INGREDIENTS

    FINISHED DOSAGE FORMULATIONS

    DIGITAL CONTENT

    GLOBAL SALES INFORMATION

    PharmaCompass
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    2D Structure
    Also known as: Revcovi, Elapegademase-lvlr, Elapegademase [inn], Unii-9r3d3y0uhs, Elapegademase [usan:inn], 9r3d3y0uhs
    Molecular Formula
    C10H20N2O5
    Molecular Weight
    248.28  g/mol
    InChI Key
    XNGDIOBPVDBNQH-QMMMGPOBSA-N
    Elapegademase is a recombinant form of the enzyme adenosine deaminase (rADA), based on the bovine amino acid sequence and covalently conjugated, with a succinimidyl carbamate linker, to monomethoxypolyethylene glycol (mPEG) (SC-PEG rADA), that can be used as an enzyme replacement agent for the treatment of adenosine deaminase severe combined immune deficiency (ADA-SCID). Upon intramuscular administration, elapegademase, as an exogenous source of ADA, replaces the deficient ADA enzyme and acts in the same manner as the endogenous enzyme. ADA is involved in purine metabolism and mainly catalyzes the irreversible hydrolytic deamination of adenosine or deoxyadenosine to inosine or deoxyinosine, respectively, as well as several naturally occurring methylated adenosine compounds. Elapegademase decreases toxic levels of adenosine and deoxyadenosine nucleotides, increases lymphocyte levels and helps maintain the proper functioning of immune cells. Elevated adenosine levels, as seen in ADA deficiency, induce apoptosis and inhibit the differentiation of thymocytes, resulting in T-lymphopenia. Low levels of deoxyadenosine and adenosine play key roles in the regulation of lymphocyte levels and the proper functioning of immune cells.
    1 2D Structure

    2D Structure

    2 Identification
    2.1 Computed Descriptors
    2.1.1 IUPAC Name
    (2S)-2-amino-6-(2-methoxyethoxycarbonylamino)hexanoic acid
    2.1.2 InChI
    InChI=1S/C10H20N2O5/c1-16-6-7-17-10(15)12-5-3-2-4-8(11)9(13)14/h8H,2-7,11H2,1H3,(H,12,15)(H,13,14)/t8-/m0/s1
    2.1.3 InChI Key
    XNGDIOBPVDBNQH-QMMMGPOBSA-N
    2.1.4 Canonical SMILES
    COCCOC(=O)NCCCCC(C(=O)O)N
    2.1.5 Isomeric SMILES
    COCCOC(=O)NCCCC[C@@H](C(=O)O)N
    2.2 Synonyms
    2.2.1 MeSH Synonyms

    1. Elapegademase-lvlr

    2. Revcovi

    2.2.2 Depositor-Supplied Synonyms

    1. Revcovi

    2. Elapegademase-lvlr

    3. Elapegademase [inn]

    4. Unii-9r3d3y0uhs

    5. Elapegademase [usan:inn]

    6. 9r3d3y0uhs

    7. Schembl20670726

    8. Ezn-2279

    9. Q27272923

    10. 1709806-75-6

    11. Poly(oxy-1,2-ethanediyl), Alpha-carboxy-omega-methoxy-, Amide With Adenosine Deaminase (synthetic)

    2.3 Create Date
    2016-08-16
    3 Chemical and Physical Properties
    Molecular Weight 248.28 g/mol
    Molecular Formula C10H20N2O5
    XLogP3-2.7
    Hydrogen Bond Donor Count3
    Hydrogen Bond Acceptor Count6
    Rotatable Bond Count10
    Exact Mass248.13722174 g/mol
    Monoisotopic Mass248.13722174 g/mol
    Topological Polar Surface Area111 Ų
    Heavy Atom Count17
    Formal Charge0
    Complexity235
    Isotope Atom Count0
    Defined Atom Stereocenter Count1
    Undefined Atom Stereocenter Count0
    Defined Bond Stereocenter Count0
    Undefined Bond Stereocenter Count0
    Covalently Bonded Unit Count1
    4 Drug and Medication Information
    4.1 Drug Indication

    Elapegademase is approved for the treatment of adenosine deaminase severe combined immune deficiency (ADA-SCID) in pediatric and adult patients. This condition was previously treated by the use of bovine pegamedase as part of an enzyme replacement therapy. ADA-SCID is a genetically inherited disorder that is very rare and characterized by a deficiency in the adenosine deaminase enzyme. The patients suffering from this disease often present with a compromised immune system. This condition is characterized by very low levels of white blood cells and immunoglobulin levels which results in severe and recurring infections.

    FDA Label

    5 Pharmacology and Biochemistry
    5.1 Pharmacology

    In clinical trials, elapegademase was shown to increase adenosine deaminase activity while reducing the concentrations of toxic metabolites which are the hallmark of ADA-SCID. As well, it was shown to improve the total lymphocyte count.

    5.2 MeSH Pharmacological Classification

    Adenosine Deaminase Inhibitors

    Drugs that inhibit ADENOSINE DEAMINASE activity. (See all compounds classified as Adenosine Deaminase Inhibitors.)

    5.3 ATC Code

    L - Antineoplastic and immunomodulating agents

    L03 - Immunostimulants

    L03A - Immunostimulants

    L03AX - Other immunostimulants

    L03AX21 - Elapegademase

    5.4 Absorption, Distribution and Excretion

    Absorption

    Elapegademase is administered intramuscularly and the reported Tmax, Cmax and AUC are approximately 60 hours, 240 mmol.h/L and 33000 hr.mmol/L as reported during a week.

    Route of Elimination

    This pharmacokinetic property has not been fully studied.

    Volume of Distribution

    This pharmacokinetic property has not been fully studied.

    Clearance

    This pharmacokinetic property has not been fully studied.

    5.5 Metabolism/Metabolites

    Metabolism studies have not been performed but it is thought to be degraded by proteases to small peptides and individual amino acids.

    5.6 Biological Half-Life

    This pharmacokinetic property has not been fully studied.

    5.7 Mechanism of Action

    The ADA-SCID is caused by the presence of mutations in the ADA gene which is responsible for the synthesis of adenosine deaminase. This enzyme is found throughout the body but it is mainly active in lymphocytes. The normal function of adenosine deaminase is to eliminate deoxyadenosine, created when DNA is degraded, by converting it into deoxyinosine. This degradation process is very important as deoxyadenosine is cytotoxic, especially for lymphocytes. Immature lymphocytes are particularly vulnerable as deoxyadenosine kills them before maturation making them unable to produce their immune function. Therefore, based on the causes of ADA-SCID, elapegademase works by supplementing the levels of adenosine deaminase. Being a recombinant and an _E. coli_-produced molecule, the use of this drug eliminates the need to source the enzyme from animals, as it was previously.