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Elapegademase

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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

Elapegademase

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 Formula	C₁₀H₂₀N₂O₅
Molecular Weight	248.28 g/mol
XLogP3	-2.7
Hydrogen Bond Donor Count	3
Hydrogen Bond Acceptor Count	6
Rotatable Bond Count	10
Exact Mass	248.13722174 g/mol
Monoisotopic Mass	248.13722174 g/mol
Topological Polar Surface Area	111 Å²
Heavy Atom Count	17
Formal Charge	0
Complexity	235
Isotope Atom Count	0
Defined Atom Stereocenter Count	1
Undefined Atom Stereocenter Count	0
Defined Bond Stereocenter Count	0
Undefined Bond Stereocenter Count	0
Covalently Bonded Unit Count	1

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.

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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.

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