Bilateral Striatal Necrosis (BSN)
Bilateral striatal necrosis (BSN) is a neurologic disorder with an intricate network of causative factors. At Protheragen, we concentrate on creating new treatment options as well as developing precise animal models to hasten preclinical studies for potential therapies for BSN. Our expertise guarantees that clients receive the most dependable and pertinent assistance, thus fast tracking the development of your medicine.
Introduction to Bilateral Striatal Necrosis (BSN)
Bilateral striatal necrosis (BSN) is a rare upper motor neuron disease characterized by symmetrical degeneration of the striatum, a key region of the basal ganglia involved in motor control, cognition, and behavior. The symptoms can differ and include muscle spasms like dystonia, speech difficulties like dysarthria, rigidity, and progressive motor function decline. More serious patients will often develop cognitive decline along with mental health conditions.
Fig.1 Diagnostic flow of bilateral striatal necrosis (BSN). (Tonduti D, et al., 2016)
Pathogenesis of Bilateral Striatal Necrosis (BSN)
In bilateral striatal necrosis (BSN), the underlying cause involves the breakdown of the mitochondria disrupting oxidative phosphorylation in striatal neurons which in turn leads to energy deficiency. The damage in the neurons is also due to excito-toxic NMDA receptors that overstimulate glutamate receptors. Infection-causing Mycoplasma pneumoniae can stimulate striatal damage due to immune response while the presence of autoantibodies that attack the basal ganglia may accelerate the damage to the neurodegenerative changes which suggests the possibility of immunomodulatory treatments in certain cases.
Fig.2 Postinfectious infantile bilateral striatal necrosis (BSN). (Tonduti D, et al., 2016)Therapeutic Development for Bilateral Striatal Necrosis (BSN)
| Drug Name | Targets | Mechanism of Action | Research Phase |
| Oral Biotin | Biotin-dependent carboxylases | Acts as a cofactor for mitochondrial enzymes, improving energy metabolism and reducing lactic acidosis in mitochondrial dysfunction-associated BSN. | Approved |
| Prednisone | Glucocorticoid receptor (GR) | Suppresses neuroinflammation and immune-mediated damage by inhibiting pro-inflammatory cytokines (e.g., IL-1β, TNF-α) and T-cell activation. | Approved |
| Intravenous Immunoglobulin (IVIg) | Fcγ receptors, complement system, autoantibodies | Neutralizes pathogenic autoantibodies, inhibits complement-mediated damage, and modulates immune cell function. | Approved |
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 upper motor neuron disease (UMND) research, Protheragen offers comprehensive preclinical services to advance bilateral striatal necrosis (BSN) 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
Diverse Therapeutic Development Platforms
Customized Drug Development Services
Disease Model Development Services
Protheragen focuses on developing multiple in vitro models of bilateral striatal necrosis (BSN), including cell-based models, brain organoids with striatal vulnerability, and microfluidic platforms that mimic neurovascular and neuroinflammatory interactions. In addition, we excel in building high-fidelity BSN animal models that accurately recapitulate disease features, allowing for robust preclinical evaluation of neuroprotective and disease-modifying therapies.
- NDUFS4 Knockout Model
- SLC19A3 Mutation Model
- NUP62 Mutation Model
- Mycoplasma Pneumoniae Infection Model
- Quinolinic Acid (QA) or Kainic Acid injection Model
At Protheragen, we are committed to validating and optimizing therapies for bilateral striatal necrosis (BSN) through preclinical studies including pharmacodynamics (PD), pharmacokinetics (PK) and toxicology to ensure their successful regulatory approval. If you are interested in our services, please feel free to contact us for more details and quotation information of related services.
Reference
- Tonduti D, Chiapparini L, Moroni I, et al. Neurological disorders associated with striatal lesions: classification and diagnostic approach[J]. Current neurology and neuroscience reports, 2016, 16: 1-15.