Schilder's Disease
Schilder's disease is a rare, aggressive form of demyelinating disorder characterized by large, confluent white matter lesions in the central nervous system. Protheragen is committed to providing cutting-edge diagnostic and therapeutic development solutions to address the challenges of Schilder's disease management. As your reliable partner in Schilder's disease therapeutic research, we provide comprehensive and high-quality services to meet all your scientific research needs.
Introduction to Schilder's Disease
Schilder's disease, also known as diffuse myelinoclastic sclerosis, is a rare and severe neuroinflammatory disorder characterized by large, confluent demyelinating lesions in the cerebral white matter. First described by Paul Ferdinand Schilder in 1912, this condition primarily affects children and young adults, though sporadic cases in older individuals have been reported.
Fig.1 Myelination is a constant process dependent on glial interaction. (Coutinho Costa V G, et al., 2023)
Pathogenesis of Schilder's Disease
The pathogenesis of Schilder's disease is primarily driven by autoimmune-mediated demyelination, where dysregulated T-cell and macrophage activity triggers inflammatory cytokine release, leading to oligodendrocyte damage and large confluent white matter lesions. Additional contributing factors include mitochondrial dysfunction in glial cells, which exacerbates oxidative stress, and potential genetic susceptibility (e.g., HLA-DRB1*15:01 allele). While the exact etiology remains unclear, viral infections (e.g., Epstein-Barr virus) may act as environmental triggers, further destabilizing myelin integrity.
Therapeutic Development for Schilder's Disease
| Drug Names | Mechanism of Action | Targets | Research Phase |
| Methylprednisolone | Potent synthetic glucocorticoid that suppresses immune-mediated inflammation by inhibiting pro-inflammatory cytokines (e.g., TNF-α, IL-6) and reducing T-cell activation. | Glucocorticoid receptor (GR), NF-κB pathway | Approved |
| Prednisone | Synthetic corticosteroid that metabolizes into prednisolone, exerting anti-inflammatory and immunosuppressive effects by blocking leukocyte infiltration and cytokine production. | Glucocorticoid receptor (GR) | Approved |
| Rituximab | Monoclonal antibody that binds to CD20 on B cells, leading to their depletion and subsequent reduction in autoantibody production and T-cell activation. | CD20 (B-cell surface antigen) | Early research |
Disclaimer: Protheragen focuses on providing preclinical research services. This table is for information exchange purposes only. This table is not a treatment plan recommendation. For guidance on treatment options, please visit a regular hospital.
Our Services
As a leader in rare neuroinflammatory diseases research, Protheragen offers comprehensive preclinical services to advance Schilder's disease therapeutics from discovery to validation. Our specialized platforms integrate genetic analysis and disease-specific modeling to replicate disease pathology with high fidelity. Using advanced blood-brain barrier (BBB) models, we optimize central nervous system (CNS) drug delivery while evaluating neuroprotection and off-target effects.
Therapeutic Development Services
By Mechanism of Action
Disease Model Development Services
In Vitro Model Development
- PLP Transgenic Model
- CNP Knockout Model
- Experimental Autoimmune Encephalomyelitis (EAE) Model
- Cuprizone-Induced Demyelination Model
- Other Models
To advance the commercialization of novel therapies for Schilder's disease, Protheragen provides comprehensive preclinical research services, covering pharmacodynamics (PD), pharmacokinetics (PK), and toxicology studies.If you are interested in our services, please feel free to contact us for more details and quotation information of related services.
Reference
- Coutinho Costa V G, Araújo S E S, Alves-Leon S V, et al. Central nervous system demyelinating diseases: Glial cells at the hub of pathology[J]. Frontiers in Immunology, 2023, 14: 1135540.