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Lead investigator

Collaborators & Co-Investigators

• Michael Berger MD, PhD

Research Sites & Affiliations

• University of British Columbia, Vancouver, British Columbia

Budget: $100,000

Disorders: Immune-Mediated Myopathies (Myositis) ,   Chronic Inflammatory demylinating polyneuropathy ,   Myasthenia Gravis

Research Areas: Understand Diagnosis and Disorder Progression

Abstract: 

One group of neuromuscular disorders are caused by inflammation, which is the body’s response to injury or external threats, like bacteria or viruses. An abnormally elevated inflammatory response, however, can result in an attack on the body’s own muscle and/or nerve cells, disrupting their normal function. Presently, individuals with inflammatory neuromuscular disease are subjected to non-specific, ineffective treatments with considerable side effects. The goal of our proposal is to identify novel, disease-modifying therapeutic targets that offer a safer, more effective alternative to alleviate disease symptoms. Granzymes are proteins that are key to the body’s normal inflammatory response. This team of scientists and physicians have shown that these granzymes are active and found at elevated levels in many inflammatory and autoimmune diseases, like skin diseases, asthma, lupus, and multiple sclerosis. Recently, other researchers have found preliminary evidence that granzymes can also be detected at high levels in some neuromuscular disease patients. In our current proposed project, we aim to comprehensively examine 3 different types of inflammatory muscular diseases to assess their granzyme levels, and their relationship to disease severity. These are Inflammatory myopathies; (Myositis; Polymositis, dermatomyositis; inclusion-body myositis); Chronic inflammatory demyelinating polyneuropathy, and Myasthenia gravis.

This study will allow us to better understand if novel therapies can be designed against granzymes to alleviate the symptoms of inflammatory neuromuscular disease.

Impact: