Stiff Person Syndrome
Stiff person syndrome (SPS) is a rare neurologic disorder characterized by muscle stiffness and spasms, leading to significant impairment in mobility and quality of life. As a leading company in the field of autoimmune diseases drug and therapy development, we are dedicated to advancing development solutions for SPS therapies.
Introduction to Stiff Person Syndrome
Stiff person syndrome (SPS), also known as stiff-man syndrome, is a neurological disorder that primarily affects the muscles and nerves involved in movement. It is characterized by debilitating muscle stiffness and spasms, often leading to difficulties in walking, standing, or performing daily activities. It is thought that the initial trigger for the development of SPS may be an autoimmune response against certain proteins found in the nerve cells, particularly the glutamic acid decarboxylase enzyme (GAD). GAD is responsible for the production of gamma-aminobutyric acid (GABA), a neurotransmitter that plays a crucial role in inhibiting muscle activity.
Fig.1 Immunopathology in stiff person syndrome. (Newsome S. D., et al., 2022)
Therapy Discovery and Development for Stiff Person Syndrome
Immunosuppressive agents such as corticosteroids, intravenous immunoglobulin (IVIG), and rituximab have been used to modulate the immune response and reduce autoantibody production in SPS cases. These therapies aim to decrease muscle stiffness and spasms by targeting the underlying autoimmune mechanism. Moreover, in severe cases of SPS that are unresponsive to conventional immunotherapies, autologous hematopoietic stem-cell transplantation (auto-HSCT) has been considered as a potential therapeutic option. This procedure involves the removal and subsequent reinfusion of the patient's own hematopoietic stem cells after high-dose chemotherapy.
At our esteemed company, we are deeply devoted to delivering cutting-edge stiff person syndrome therapeutic development services. If you are intrigued by our offerings and would like to explore further, we warmly invite you to click on the link below to gain deeper insights into our comprehensive range of services.
Our Services
As a leading company in autoimmune diseases drug and therapy development, our company offers comprehensive services for the development of diagnostics and novel therapies for stiff person syndrome. Our expertise and capabilities in SPS therapy development include preclinical research, animal model and in vitro model development.
To develop the anti-glutamic acid decarboxylase (GAD) antibody injection model, we carefully select animal subjects, typically white mice, and perform intracerebral injections of anti-GAD antibodies that are precipitated from the blood of stiff person syndrome cases.
Anti-GAD Antibody Injection Models
We carefully select appropriate cell lines that possess relevant characteristics and functionalities for cell-based model development. These cell lines may include neural cells, such as primary neurons or immortalized cell lines, as well as other relevant cell types, including immune cells and glial cells.
Our organoid models for SPS involve the cultivation of patient-derived cells or induced pluripotent stem cells (iPSCs) in a specialized culture system that supports the development of organoid structures. These organoids can be derived from neural tissues, such as the brain or spinal cord.
Through rigorous experimental design and execution, we conduct in-depth evaluations of potential therapeutic candidates, including immunomodulatory agents, gene therapies, and novel drug delivery systems. Beyond the wide range of services and models mentioned previously, our expertise extends to crafting bespoke solutions and designing disease models that are perfectly tailored to your individual requirements. We take great pride in our ability to adapt and customize our offerings to meet the unique needs of each client. If our comprehensive range of services has piqued your interest, we wholeheartedly encourage you to contact us without any hesitation.
References
- Newsome Scott D., and Tory Johnson. "Stiff person syndrome spectrum disorders; more than meets the eye." Journal of neuroimmunology 369 (2022): 577915.
- Dalakas Marinos C. "Therapies in stiff-person syndrome: advances and future prospects based on disease pathophysiology." Neurology: Neuroimmunology & Neuroinflammation 10.3 (2023): e200109.