First Indication for Lead TREM2 Agonist Program Announced as Adult-onset Leukoencephalopathy with Axonal Spheroids and Pigmented Glia (ALSP)

Financing Led by Vida Ventures; Stefan Vitorovic, Co-founder and Managing Director, to Join Board of Directors

CAMBRIDGE, MA, USA I August 18, 2021 I Vigil Neuroscience, a biotechnology company harnessing the power of microglia for the treatment of neurodegenerative diseases, today announced the completion of a $90 million Series B financing to further advance Vigil’s proprietary pipeline of microglia-targeted medicines for the treatment of neurodegeneration. The financing was led by Vida Ventures with participation from existing investors Atlas Venture, Northpond Ventures and Hatteras Venture Partners as well as new investors including Surveyor Capital (a Citadel company), Cormorant Asset Management, Invus, OrbiMed, Rock Springs Capital, Deep Track Capital, Logos Capital, Pivotal bioVenture Partners, and Lightstone Ventures.

Vigil is developing both a fully human monoclonal antibody, VGL101, and small molecule agonists of triggering receptor on myeloid cells 2 (TREM2), an essential microglia sensor that mediates responses to environmental signals to maintain brain homeostasis. TREM2 is a compelling molecular target for neurodegeneration as it serves as a damage sensor of microglia with trophic function and plays a role in microglia response to CNS injury. The company expects to initiate a Phase 1 study to evaluate VGL101 in healthy volunteers on safety, pharmacokinetics and pharmacodynamics by year end 2021.

The first indication for VGL101 will be adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP), a rare inherited neurodegenerative disease caused by a mutation to the CSF1R gene for which there are no therapies currently approved by the FDA. VGL101 has the potential to address an estimated 10,000 people in the United States, with similar prevalence in Europe and Japan, living with this devastating disease. Microglia dysfunction is central to ALSP pathogenesis and Vigil believes TREM2 agonism with VGL101 can restore microglia function, thereby potentially providing therapeutic benefits to patients with the disease. ALSP represents the first indication in Vigil’s precision medicine strategy of applying learnings from rare indications with strong genetic, biochemical and pathophysiological associations to microglial deficiency to the development of microglia-based therapeutics in more common indications such as Alzheimer’s Disease. The company is planning to initiate a natural history study enrolling ALSP patients this fall to better understand disease characteristics and evaluate fluid and imaging biomarkers.

“ALSP is a devastating disease that has a strong genetic link to microglia dysfunction and signaling deficiency. We plan to work closely with patients and their families to unravel the complexities of the disease and rapidly advance VGL101 through the clinic,” said Ivana Magovčević-Liebisch, PhD, JD, President and Chief Executive Officer of Vigil. “This financing will enable us to accelerate both our lead TREM2 activating monoclonal antibody in patients as well as advance our small molecule program through important milestones. The remarkable progress our team has made is a testament to our commitment to creating a better tomorrow for patients with neurodegenerative diseases. I applaud their efforts.”

Vigil is also performing lead optimization in its small molecule program to develop novel first-in-class agonists of human TREM2 with a compelling profile of potency, solubility, oral bioavailability and CNS uptake. The company plans to apply learnings from its precision-based approach to the development of small molecule TREM2 agonists for more common neurodegenerative diseases such as Alzheimer’s Disease for which oral administration and high CNS penetrance can meaningfully impact disease treatment.

In conjunction with the closing of the financing, Stefan Vitorovic, Co-founder and Managing Director of Vida Ventures, will join the Vigil Board of Directors. “We are delighted to lead this financing with an outstanding group of investors and world-class team. Our research has led us to conclude that there is substantial biological evidence to suggest that restoring the function of microglia plays a key role in arresting neurodegeneration,” commented Mr. Vitorovic. “Vigil is at the forefront of pioneering such innovative treatments, and I am excited about the potential of their novel precision medicine-based strategy to rapidly bring impactful medicines to patients.”

“We look forward to working with both the team from Vida as well as all our new and existing investors as we continue to build Vigil into a leading precision medicine company focused on microglia,” said Bruce Booth, DPhil, Chairman of the Board of Vigil. “We are fortunate to have these leading investors supporting our efforts, and we welcome Stefan to our Board.”

About ALSP

Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a rare, inherited, autosomal dominant neurological disease with high penetrance. It is caused by a mutation to the CSF1R gene and affects an estimated 10,000 people in the US, with about 1,000 new cases annually. The disease generally presents itself in the fourth decade of life, is diagnosed through genetic testing and established clinical/radiologic criteria and is characterized by cognitive dysfunction, neuropsychiatric symptoms, and motor impairment. These symptoms typically exhibit rapid progression with a life expectancy of approximately 7 years on average after diagnosis, causing significant patient and caregiver burden. There are currently no approved products for the treatment of ALSP, underlining the high unmet need in this rare indication. Patients and caregivers can find more information on ALSP at

About Vigil Neuroscience

Vigil Neuroscience is a microglia-focused therapeutics company treating both rare and common neurodegenerative diseases by restoring the vigilance of microglia, the sentinel cells of the brain’s immune system. We are utilizing the tools of modern neuroscience drug development across multiple therapeutic modalities to rapidly deliver precision-based therapies to improve the lives of patients and their families.

SOURCE: Vigil Neuroscience