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Myasthenia Gravis

Myasthenia gravis (MG) is classified within a group of neurological disorders characterized by impaired signal transmission between nerves and muscles, leading to significant muscle fatigue. These conditions are collectively known as neuromuscular transmission disorders or myasthenic syndromes. MG is a non-hereditary autoimmune disease in which there is a dysfunction at the motor endplate of skeletal muscle, the exact cause of which remains not fully understood. The clinical presentation of myasthenia gravis is marked by muscle weakness that is dependent on exertion. This weakness often affects the aforementioned muscles asymmetrically and can impact single or multiple muscles, irrespective of the side of the body. antibodies-online offers antibodies and proteins for key MG targets which support development of novelle therapeutics.

What is Myasthenia Gravis?

MG is an autoimmune neuromuscular disorder characterized by fluctuating muscle weakness and fatigue. The disease is primarily caused by autoantibodies targeting components of the neuromuscular junction, notably the acetylcholine receptor (AChR) and muscle-specific kinase (MuSK). The pathophysiology of MG involves a complex interplay of genetic predisposition and environmental factors, leading to an aberrant immune response.

What Antigens are Involved in Myasthenia Gravis Pathogenesis?

Key targets in MG are the nicotinic acetylcholine receptors (CHRN), which are targeted by autoantibodies. The autoantibodies lead to receptor internalization and complement-mediated damage. Search for other pathogenic antigens has detected antibodies against the muscle-specific tyrosine kinase MuSK and low-density lipoprotein-related protein 4 (Lrp4), both causing pre- and post-synaptic impairments. The muscle membrane-bound protein agrin (heparin sulfate proteoglycan) is considered as fourth pathogen however its role is not fully understood yet.

Human Leukocyte Antigen Targets in MG

The HLA system is crucial for immune function, and specific HLA alleles are involved in presenting autoantigens to the immune system, leading to the activation of autoreactive T cells. These activated T cells can then help B cells produce autoantibodies against components of the neuromuscular junction, such as the acetylcholine receptor or muscle-specific kinase. HLA-DRB1*03:01 for example presents autoantigens to CD4+ T cells, facilitating an autoimmune response against AChR. Similar to HLA-DRB1*03:01, HLA-DRB1*16: helps in presenting autoantigens to T cells, promoting autoimmunity in MuSK-positive MG. HLA-DQB1*05:02: helps in presenting autoantigens related to MuSK, thus contributing to the autoimmune response in these patients.

Product
Source
Application
Cat. No.
Quantity
Delivery
Datasheet
Source Mammalian Cells
Application BP, CP, ELISA, Neut
Cat. No. ABIN7273302
Quantity 100 μL
Delivery 17 to 20 Days
Datasheet Datasheet
Source Mammalian Cells
Application BP, CP, ELISA, Neut
Cat. No. ABIN7273254
Quantity 100 μL
Delivery 17 to 20 Days
Datasheet Datasheet
Source Mammalian Cells
Application BP, CP, ELISA, Neut
Cat. No. ABIN7273232
Quantity 100 μL
Delivery 17 to 20 Days
Datasheet Datasheet

Therapeutic Approaches for MG

Current therapeutic interventions for MG range from symptomatic treatments to immunomodulatory therapies. Symptomatic treatments, such as acetylcholinesterase inhibitors, enhance neuromuscular transmission by increasing the availability of acetylcholine at the neuromuscular junction. Immunomodulatory therapies include corticosteroids and other immunosuppressants, which reduce overall immune activity. Recently, targeted therapies that interfere with the autoimmune response have gained prominence. Emerging therapies specifically targeting FcRn, such as efgartigimod and rozanolixizumab, represent a novel approach in MG management. These agents are designed to decrease pathogenic IgG levels by blocking FcRn, thereby facilitating the degradation of IgG and reducing autoantibody-mediated pathogenicity.

Antibodies for MG Research

antibodies-online offers high quality anti-FcRn antibodies for MG Research. The antibodies can block the IgG binding site and therfore support development of novelle therapeutics. They are thoroughly characterized, including binding sites, binding kinetics, species cross reactivities and applications. Their binding and block capacities were verified in mouse models and assured via two negative controls.

FcRn Antibody (ADM31)
  • Blocks Human Serum Albumin
  • Works in FACS, IF, WB
  • Recognizes Human FcRn
FcRn Antibody (DVN24)
  • Blocks Human & Mouse IgG
  • Works in FACS, IF
  • Recognizes Human & Mouse FcRn

Another notable approach to combat MG is eculizumab, a monoclonal antibody that inhibits complement activation and prevents damage to the neuromuscular junction. The production of these biotherapeutics involves sophisticated techniques, including recombinant DNA technology and cell culture systems to ensure high specificity and efficacy. The manufacturing process for FcRn inhibitors typically involves the expression of the therapeutic protein in mammalian cell lines, followed by purification and formulation to meet regulatory standards for clinical use.

Available Biosimilar Antibodies for MG Targets

Product
Reactivity
Clonality
Application
Cat. No.
Quantity
Reactivity Human
Clonality Monoclonal
Application FACS, in vivo
Cat. No. ABIN7200672
Quantity 1 mg
Reactivity Human
Clonality Monoclonal
Application
Cat. No. ABIN7487797
Quantity 100 μg
Reactivity Human
Clonality Chimeric
Application ELISA, WB
Cat. No. ABIN7538074
Quantity 100 μg
Reactivity Human
Clonality Monoclonal
Application
Cat. No. ABIN7488315
Quantity 100 μg
Reactivity Human
Clonality Monoclonal
Application
Cat. No. ABIN7488229
Quantity 100 μg

In conclusion, advancing our understanding of the immunological underpinnings of MG and leveraging this knowledge to develop targeted therapeutics is paramount. Continued research into the specific antigens and immune interactions involved in MG will facilitate the development of next-generation therapies that can improve patient outcomes and quality of life.


References

  1. Takamori: "Myasthenia Gravis: From the Viewpoint of Pathogenicity Focusing on Acetylcholine Receptor Clustering, Trans-Synaptic Homeostasis and Synaptic Stability." in: Frontiers in molecular neuroscience, Vol. 13, pp. 86, (2020) (PubMed).
  2. Zhu, Hou, Wang, Zhang, Wang, Guo, Wu: "FcRn inhibitors: a novel option for the treatment of myasthenia gravis." in: Neural regeneration research, Vol. 18, Issue 8, pp. 1637-1644, (2023) (PubMed).
  3. Bhandari, Bril: "FcRN receptor antagonists in the management of myasthenia gravis." in: Frontiers in neurology, Vol. 14, pp. 1229112, (2023) (PubMed).
  4. Narayanaswami, Sanders, Wolfe, Benatar, Cea, Evoli, Gilhus, Illa, Kuntz, Massey, Melms, Murai, Nicolle, Palace, Richman, Verschuuren: "International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update." in: Neurology, Vol. 96, Issue 3, pp. 114-122, (2021) (PubMed).
  5. Cavalcante, Mantegazza, Antozzi: "Targeting autoimmune mechanisms by precision medicine in Myasthenia Gravis." in: Frontiers in immunology, Vol. 15, pp. 1404191, (2024) (PubMed).
Julian Pampel
Julian Pampel, BSc
Content Manager at antibodies-online.com

Creative mind of antibodies-online with a keen eye for details. Proficient in the field of life-science with a passion for plant biotechnology and clinical study design. Responsible for illustrated and written content at antibodies-online as well as supervision of the antibodies-online scholarship program.

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