Progressive Bulbar Palsy (PBP)
Progressive bulbar palsy (PBP) treatment is complex because of fast progression, absence of effective disease-modifying therapies, and a requirement for multidisciplinary approach to care for complex symptoms such as dysphagia and breathing problems. Given our strong knowledge on PBP therapy development, Protheragen is positioned to offer custom solutions and complete aids to help you transition from PBP therapy research to commercialization.
Introduction to Progressive Bulbar Palsy (PBP)
Progressive bulbar palsy (PBP) is a form of a severe neurodegenerative disorder and falls within the category of motor neuron diseases (MNDs). Its central pathology is located in the bulbar region of the brainstem. PBP is particularly relentless because the affected brainstem region contains motor nuclei for the speech, swallowing, and mastication functions. Unlike classic amyotrophic lateral sclerosis (ALS), which entails upper and lower motor neuron lesions, PBP early in its course is biased toward lower motor neuron (LMN) lesions, although most cases eventually transition to a generalized ALS phenotype.
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Fig.1 Amyotrophic lateral sclerosis (ALS) phenotypic spectrum. (Ilieva H, et al., 2023)
Pathogenesis of Progressive Bulbar Palsy (PBP)
The mediating factors of TDP-43 proteinopathy, mitochondrial dysfunction, and glutamate mediated excitotoxicity lead to the lower motor neuron degeneration in bulbar region of brain stem with progressive bulbar palsy (PBP). Activated microglia and astrocytes induce neuroinflammation parallel to the weakening of the nervous system, which is the result of proinflammatory cytokines. Neurologically genetic such as C9ORF72 hexanucleotide expansion and SOD1 mutation , along with some environmental factors, may impact the development of a disorder.
Therapeutic Development for Progressive Bulbar Palsy (PBP)
| Drug Names | Mechanism of Action | Targets | Research Phase |
| Tannic acid | Antioxidant and anti-inflammatory properties; may reduce oxidative stress in motor neurons. Potential modulation of protein aggregation (e.g., TDP-43). | Reactive oxygen species (ROS), TDP-43 aggregates | Approved |
| Benzocaine | Local anesthetic that blocks voltage-gated sodium channels, potentially reducing neuropathic pain or hyperexcitability in bulbar neurons. | Voltage-gated sodium channels | 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
Protheragen is dedicated to the diagnostic and therapeutic development services of rare lower motor neuron diseases (LMND) such as progressive bulbar palsy (PBP). We prioritize the creation of sophisticated models of disease mechanisms and employ novel blood-brain barrier (BBB) models to improve therapeutic agent delivery and action. We strive towards improving the development of precise diagnostic and effective therapeutic interventions for PBP through our thorough strategies.
Therapeutic Development Services
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By Mechanism of Action
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Disease Model Development Services
- SOD1-G93A Overexpression Model
- SOD1-G37R Overexpression Model
- TDP-43 Transgenic Model
- C9ORF72 Repeat Expansion Model
- Ricin/Botulinum Induced Model
- Other Models
Focusing on preclinical research, Protheragen offers comprehensive pharmacodynamic (PD), pharmacokinetic (PK), and toxicology study services to support the development and regulatory approval of potential therapies. If you are interested in our services, please feel free to contact usfor more details and quotation information of related services.
Reference
- Ilieva H, Vullaganti M, Kwan J. Advances in molecular pathology, diagnosis, and treatment of amyotrophic lateral sclerosis[J]. bmj, 2023, 383.