BIIB092 is a product that is being investigated for the treatment of progressive supranuclear palsy (PSP). PSP is a rare condition that is a result of destruction of nerve cells in certain parts of the brain causing problems with balance, movement, vision, speech and swallowing. In patients with PSP, an abnormal form of a protein called tau accumulates in specific areas of the brain by spreading from brain cell to brain cell leading to their damage. Over time, PSP gets progressively worse, with people becoming severely disabled within three to five years of onset. Currently, there is no cure for PSP and no treatment to slow down the disease.
BIIB092 is a monoclonal antibody that acts by recognising, binding to and neutralising a specific type of tau protein responsible for the nerve cells damage and gradual worsening of PSP. This action of BIIB092 is thought to prevent the spread of the disorder and potentially slow the progression of PSP. Tau-directed immunotherapy like BIIB092 offers a promising disease-modifying treatment strategy for PSP and similar conditions. BIIB092 is given by intravenous infusion and if licensed, will offer a more targeted treatment option for patients with PSP.
Tofersen (BIIB067) is in clinical development for the treatment of amyotrophic lateral sclerosis (ALS – also known as motor neurone disease) caused by mutations in the SOD1 gene (SOD1-ALS). ALS is a progressive disease of the nervous system, where nerve cells in the brain and spinal cord that control voluntary movement gradually deteriorate, causing loss of muscle function and paralysis. ALS is a debilitating and life-threatening disease. The gradual loss of neurons leads to a paralysing effect on muscles used for breathing, which usually leads to death from respiratory failure.